LIP PAPULES

Basal cell carcinoma, pigmented Kaposi’s sarcoma Rook p.3130, 1998, Sixth Edition Labial melanotic macule (benign labial lentigo) Oral Surg Oral Med Oral Pathol 42:192-205, 1976 Lentigo maligna – hyperpigmented lips and oral mucosa AD 138:1216-1220, 2002 Lentigo maligna melanoma Lentigo simplex Leukoplakia Rook p.3130, 1998, Sixth Edition Melanocytic nevus, junctional Melanoma BJD 152:1324-1326, 2005 Seborrheic keratosis

Ephelide Rook p.3131, 1998, Sixth Edition Lichenoid photodermatitis Solar lentigo

Acanthosis nigricans JAAD 31:1-19, 1994 Fordyce spots – yellow macule BJD 129:355, 1993; BJD 121:669-670, 1989 Lichen planus Vitiligo – repigmenting

Albright’s syndrome Cronkhite-Canada syndrome Hereditary acrolabial telangiectasia – blue lips, areolae, and nail bed, telangiectasias Idiopathic lenticular pigmentation – oral, labial, perianal, and digital pigmented macules AD 132:844-845, 1996 LAMB/NAME syndrome (Carney complex) Textbook of Neonatal Dermatology, p.371, 2001; Oral Surg 63:175-183, 1987; Medicine 64:270-283, 1985 Laugier-Hunziker syndrome AD 132:844-845, 1996; Dermatologica 181:183-186, 1990 LEOPARD syndrome Textbook of Neonatal Dermatology, p.371, 2001 Neurofibromatosis Peutz-Jeghers syndrome JAAD 53:660-662, 2005; Curr Prob Derm 14:41-70, 2002; Rook p.3063, 1998, Sixth Edition; J Dent Child 44:131-134, 1977; J Periodontol 42:726-736, 1971

Arsenic poisoning – chronic BJD 149:757-762, 2003 Heavy metal poisoning

Venous hemangioma Venous lake

CREST syndrome Lupus erythematosus – bullous LE, discoid lupus erythematosus Rook p.2444-2449, 1998, Sixth Edition; NEJM 269:1155-1161, 1963

Congenital rhabdomyomatous mesenchymal hamartoma (striated muscle hamartoma) – skin-colored pedunculated (polypoid) nodule of neck, midline of chin, upper lip Ped Derm 16:65-67, 1999; Ped Derm 7:199-204, 1990; Ped Derm 3:153-157, 1986 Sucking calluses Textbook of Neonatal Dermatology, p.478, 2001

Cosmetic tattoos – sarcoid AD 141:869-872, 2005

Granuloma inguinale – extragenital lesions of nose and lips, or extremities; papule or nodule breaks down to form ulcer with overhanging edge; deep extension may occur; or serpiginous extension with vegetative hyperplasia; pubis, genitalia, perineum JAAD 32:153-154, 1995; JAAD 11:433-437, 1984 Leishmaniasis Leprosy – lepromatous leprosy Rook p.1224, 1998, Sixth Edition; histoid nodules of the lip Int J Lepr Other Mycobact Dis 65:374-375, 1997 Molluscum contagiosum J Oral Med 35:62-64, 1980 Paracoccidioidomycosis Pinta Rhinoscleroma – plaques of nodules of upper lip Rook p.3135, 1998, Sixth Edition Syphilis, primary (chancre) – ulcerated plaque; secondary – split papules, nodular secondary syphilis Br Dent J 160:237-238, 1986 Verruca vulgaris Tyring p.261,272, 2002; Oral Surg Oral Med Oral Pathol 62:410-416, 1986 Yaws

Amyloidosis, primary systemic Lichen myxedematosus Mucocoele Xanthoma disseminatum (Montgomery’s syndrome) – red-yellow-brown lip papules NEJM 338:1138-1143, 1998; JAAD 23:341-346, 1990; AD Syphilol 37:373-402, 1938

Cheilitis glandularis Granulomatous cheilitis Lymphocytoma cutis JAAD 38:877-905, 1998 Necrotizing sialometaplasia J Oral Maxillofac Surg 58:1419-1421, 2000 Sarcoidosis Bolognia p.1457, 2003

Milia-like calcinosis cutis Ped Derm 21:483-485, 2004

Actinic keratosis Rook p.1671, 1998, Sixth Edition Adenolipoma BJD 148:606-607, 2003 Apocrine gland carcinoma Oral Surg Oral Med Oral Pathol Oral Radiol Endod 82:93-99, 1996 Basal cell carcinoma Rook p.1681-1683, 1998, Sixth Edition; Acta Pathol Mibrobiol Scand 88A:5-9, 1980 Chondroid syringoma – upper lip Cutis 71:49-55, 2003 Cutaneous horn Epidermal nevus Fibroma (irritation fibroma) Rook p.3130, 1998, Sixth Edition Fordyce spots – ectopic sebaceous glands; yellow papules of buccal mucosa, lip, penile shaft Textbook of Neonatal Dermatology, p.479, 2001; Rook p.1982, 1998, Sixth Edition Granular cell tumor Histiocytosis Juvenile xanthogranuloma Kaposi’s sarcoma Rook p.3130, 1998, Sixth Edition; Oral Surg Oral Med Oral Pathol 71:38-41, 1991 Keratoacanthoma Oral Surg Oral Med Oral Pathol 38:918-927, 1974 Leiomyoma Lymphoma Oral Oncol 29B:225-230, 1993; histiocytic lymphoma Lymphomatoid papulosis – ulcerated papule JAAD 44:339-341, 2001 Melanocytic nevus Rook p.1722-1723, 1998, Sixth Edition Melanoma – desmoplastic melanoma of lip – ulcerated nodule, lip ulcer, induration and edema JAAD 47:863-868, 2002 Merkel cell carcinoma – reddish-blue nodules; legs, lip, eyelid, scalp, nose Histopathology 7:229-249, 1983 Microcystic adnexal carcinoma JAAD 45:283-285, 2001; JAAD 41:225-231, 1999 Mucocele AD 101:673-678, 1970 Mucosal neuroma Multinucleate cell angiohistiocytoma Cutis 59:190-192, 1997; Oral Surg Oral Med Oral Pathol 78:743-747, 1994 Papillary syringoadenoma (syringocystadenoma papilliferum) Rook p.3145, 1998, Sixth Edition Plasma-acanthoma of the lip Rook p.3142, 1998, Sixth Edition Plasmacytoma JAAD 35:777-778, 1996; JAAD 14:335-339, 1986 Reticulohistiocytoma AD 139:381-386, 2003; JAAD 46:801, 2002 Rhabdomyoma Am J Dermatopathol 22:264-267, 2000 Rhabdomyosarcoma, congenital – lip nodule Ped Derm 20:335-338, 2003 Sclerotic fibroma – labial mucosal papulonodule BJD 149:433-435, 2003 Sebaceous adenoma Rook p.1722-1723, 1998, Sixth Edition Sebaceous carcinoma JAAD 48:401-408, 2003 Spindle cell carcinoma Spindle cell hemangioendothelioma Rook p.3130, 1998, Sixth Edition

Squamous cell carcinoma Rook p.1689-1690,3074-3076, 1998, Sixth Edition; Otolaryngol Clin North Am 26:265-277, 1993 Traumatic neuroma Trichoblastic carcinoma Derm Surg 27:663-666, 2001 Trichoepitheliomas BJD 4:269-286, 1892 Trichilemmomas Verrucous carcinoma – arising in lichen planus Cutis 65:219-222, 2000 Verrucous xanthoma AD 136:665-670, 2000

Actinic lichen planus JAAD 20:226-231, 1989

Acanthosis nigricans (cobblestoning) Rook p.1584, 1998, Sixth Edition Granuloma annulare Lichen planus BJD 132:1000-1002, 1995 Reactive perforating collagenosis JAAD 25:1079-1081, 1991

Factitial dermatitis

Blue rubber bleb nevus syndrome – vascular malformation AD 116:924-929, 1996 Cowden’s syndrome – lip papillomas BJD 148:1040-1046, 2003; Rook p.3129, 1998, Sixth Edition Focal epithelial hyperplasia (Heck’s disease) Tyring, p.273, 2002; BJD 96:375-380, 1977 Focal palmoplantar and oral mucosa (gingival) hyperkeratosis syndrome (MIM:148730) – palmoplantar keratoderma, leukoplakia, and cutaneous horn of the lips BJD 146:680-683, 2002 Ganglioneuromatosis type III multiple endocrine neoplasia (MEN) JID November 1994 Goltz’s syndrome (focal dermal hypoplasia) – asymmetric linear and reticulated streaks of atrophy and telangiectasia; yellow-red nodules; raspberry-like papillomas of lips, perineum, acrally, at perineum, buccal mucosa; xerosis; scalp and pubic hair sparse and brittle; short stature; asymmetric face; syndactyly, polydactyly; ocular, dental, and skeletal abnormalities with osteopathia striata of long bones JAAD 25:879-881, 1991 Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease) Rook p.2091, 1998, Sixth Edition; Am J Med 82:989-997, 1987 Juvenile hyaline fibromatosis (infantile systemic hyalinosis) – nodular perianal lesions, ears, lips, gingival hypertrophy, hyperpigmentation, flexion contractures of joints, osteolytic defects, stunted growth Dermatology 190:148-151, 1995; Ped Derm 11:52-60, 1994 Lesch-Nyhan syndrome Lipoid proteinosis Int J Derm 39:203-204, 2000; Acta Paediatr 85:1003-1005, 1996; JAAD 27:293-297, 1992 Multiple endocrine neoplasia syndrome (MEN I) – angiofibromas AD 133:853-857, 1997 Multiple mucosal neuroma syndrome (MEN IIB) – skin-colored papules and nodules of lips, tongue, oral mucosa JAAD

36:296-300, 1997; NEJM 335:943-951, 1996; Oral Surg 51:516-523, 1981; J Pediatr 86:77-83, 1975; Am J Med 31:163-166, 1961 Multicentric reticulohistiocytosis – digital papule; knuckle pads yellow papules and plaques Rook p.2325-2326, 1998, Sixth Edition; AD 126:251-252, 1990; Oral Surg Oral Med Oral Pathol 65:721-725, 1988; Pathology 17:601-608, 1985; JAAD 11:713-723, 1984; AD 97:543-547, 1968 Neurofibromatosis Nevoid basal cell carcinoma syndrome Trichorhinophalangeal syndrome I – autosomal dominant; pearshaped nose, tubercle of normal skin below the lower lip, fusiform swelling of the PIP joints; fine brittle sparse hair, eyebrows sparse laterally, dense medially, short stature JAAD 31:331-336, 1994

Sucking callus (sucking pads) – in neonates Ped Derm 4:123-128, 1987

Acquired progressive lymphangioma Rook p.3130, 1998, Sixth Edition Angiofibroma Angiolymphoid hyperplasia with eosinophilia BJD 145:365, 2001; BJD 134:744-748, 1996 Angiosarcoma Rook p.3130, 1998, Sixth Edition Arteriovenous fistula (traumatic) Cutis 62:235-237, 1998 Caliber-persistent arteries of the lip JAAD 46:256-259, 2002 Epithelioid hemangioendothelioma JAAD 36:1026-1028, 1997 Epithelioid hemangioma Rook p.3130, 1998, Sixth Edition Hemangioma Otolaryngol Clin North Am 19:769-796, 1986; focal hemangioma AD 139:869-875, 2003 Hemangiopericytoma Intravascular papillary endothelial hyperplasia (Masson’s hemangioma) Cancer 38:1227-1236, 1976 Lymphatic malformation Pyogenic granuloma Rook p.2354-2355, 1998, Sixth Edition; Am J Surg Pathol 4:471-479, 1980 Venous lake Clin Exp Dermatol 15:115-118, 1990 Wegener’s granulomatosis JAAD 49:335-337, 2003

LIP PITS Angular sinuses Commissural lip pits – autosomal dominant; usually bilateral may be associated with pre-auricular sinuses or pits Oral Surg Oral Med Oral Pathol 21:56-60, 1966; may communicate with parotid duct Arch Otolaryngol Head Neck Surg 116:1445-1447, 1990 Congenital sinus of the lower lip (with lower lip pits) (congenital fistulae of lower lip) – bilateral Textbook of Neonatal Dermatology, p.478, 2001; Trans St John’s Hosp Dermatol Soc 61:82-86, 1975 Congenital lower lip pits Ped Derm 19:363-364, 2002; Ped Derm 15:443-445, 1998; Cutis 61:127-128, 1998; JAAD 32:520-521, 1995; Am J Hum Genet 6:244-256, 1954; may

communicate with underlying salivary glands Rook p.3126-3127, 1998, Sixth Edition Congenital conductive or mixed deafness, preauricular sinus, external ear anomaly, commissural lip pits – autosomal dominant Ann Otol Rhinol Laryngol 100:928-932, 1991 Focal dermal hypoplasia, morning glory anomaly, and polymicrogyria – swirling pattern of hypopigmentation, papular hypopigmented and herniated skin lesions of face, head, hands, and feet, basaloid follicular hamartomas, mild mental retardation, macrocephaly, microphthalmia, unilateral morning glory optic disc anomaly, palmar and lip pits, and polysyndactyly Am J Med Genet 124A:202-208, 2004 Hirschsprung’s disease with cleft palate and paramedian pits of the lower lip J Pediatr Surg 14:162-164, 1979 Kabuki make-up syndrome (Niikawa-Kuroki syndrome) – lower lip pits and anorectal anomalies Am J Med Genet 86:282-284, 1999 Median cleft of the lower lip with lip pits and clefts of the lip and palate Cleft Palate Craniofac J 36:86-87, 1999 Midline sinus of the upper lip Plast Reconstr Surg 65:674-675, 1980 Orofacial digital syndrome type 1 – lower lip pits, oral frenula, and clefts, hypoplasia of the nasal cartilage, malformation of the hands, hypertelorism, mental retardation Cutis 61:127-128, 1998 Popliteal pterygium syndrome – lower lip pits, oral clefts, pterygium of the leg, genitourinary anomalies, congenital heart disease, syndactyly Ped Derm 15:443-445, 1998 Trichorrhexis nodosa with lip pits – autosomal dominant ectodermal dysplasia with central nervous system malformations Am J Med Genet 71:226-228, 1997 Van der Woude syndrome – autosomal dominant; lower lip pits, cleft lip, palate, and uvula, hypodontia; mutation of interferon regulatory factor 6 (IRF6) NEJM 351:769-780, 2004; Ped Derm 15:443-445, 1998

Allergy to food additives, cobalt AD 129:477, 1993 Allergic contact dermatitis to many exogenous agents – oils, waxes, antioxidants; flavorings Rook p.757, 1998, Sixth Edition; latex balloons Cutis 54:138-140, 1994; toothpaste Contact Dermatitis 28:42, 1993; contact orofacial granulomatosis to gold and mercury JAAD 49:1117-1120, 2003; epoxy diacrylates JAAD 38:116-120, 1998; collophonium in dental floss Comm Dent Oral Epidemiol 19:155-159, 1990; toothpaste materials Comm Dent Oral Epidemiol 19:155-159, 1990; oral allergy syndrome – avocado, banana, celery, eggplant, jackfruit, kiwi, mango, strawberry JAAD 53:736-737, 2005 Angioedema JAAD 25:155-161, 1991; Dermatol Clin 3:85-95, 1985 Chronic granulomatous disease – cheilitis JAAD 36:899-907, 1997; JAAD 23:444-450, 1990 Lupus erythematosus, systemic BJD 121:727-741, 1989; bullous lupus erythematosus Cutis 70:31-34, 2002 Myeloperoxidase deficiency – lip infection and necrosis Ped Derm 20:519-523, 2003 Pemphigus vegetans, Neumann variant JAAD 39:872-875, 1998

Pemphigus vulgaris Scleroderma Sjögren’s syndrome – red swollen lip J Dermatol 28:47-49, 2001 Urticaria JAAD 50:815-817, 2004

Double lip Cutis 66:253-254, 2000 Sucking calluses Textbook of Neonatal Dermatology, p.479, 2001

Mucous cyst (mucocele)

ACE-inhibitor induced angioedema J Forensic Sci 46:1239-1243, 2001 Fixed drug eruption Tacrolimus, systemic – fibromatosis Hypersensitivity reactions

Aquagenic angioedema Cutis 37:465-466, 1986 Abreva Antioxidants Comm Dent Oral Epidemiol 19:155-159, 1990 Benzoates Comm Dent Oral Epidemiol 19:155-159, 1990 Chironoida larvae used as fish food – angioedema Acta DV 78:482-483, 1998 Chocolate – granulomatous cheilitis BJD 150:595, 2004 Flavorings Comm Dent Oral Epidemiol 19:155-159, 1990 Monosodium glutamate-related orofacial granulomatosis Oral Surg Oral Med Oral Pathol 71:560-564, 1991 Silica granuloma – mimics granulomatous cheilitis Dermatologica 181:246-247, 1990 Sorbic acid – non-allergic urticaria Ghatan p.187, 2002, Second Edition Tattooing, cosmetic AD 141:918-919, 2005

Anthrax Ped Derm 18:456-457, 2001; Rook p.3140, 1998, Sixth Edition Cancrum oris (noma) – labial and buccal necrosis J Dent Child 48:138-141, 1981 Candidiasis – chronic mucocutaneous candidiasis Cysticercosis Br Dent J 170:105-106, 1991 Dentoalveolar abscess JAAD 50:815-817, 2004 Diphtheria Rook p.3140, 1998, Sixth Edition Elephantiasis nostras of the lips – presumed streptococcal infection Oral Surg Oral Med Oral Pathol Endod 84:297-300, 1997 Epstein-Barr virus – swollen erythema of face with swollen lips BJD 143:1351-1353, 2000 Erysipelas Furuncle Rook p.3140, 1998, Sixth Edition

Hand foot and mouth disease (Coxsackie virus) Rook p.3133-3134, 1998, Sixth Edition Herpes simplex infection Rook p.3133-3134, 1998, Sixth Edition; primary herpetic gingivostomatitis Tyring p.75, 2002 Herpes zoster Impetigo Insect bite reaction Larva migrans Leishmaniasis Mycoses 41 Suppl2:78-80, 1998 (German); JAAD 28:495-496, 1993; Dermatologica 150:292-294, 1975; Leishmania aethiopica – lip edema Trans R Soc Trop Med Hyg 63:708-737, 1969; espundia (mucocutaneous leishmaniasis) – nasopharyngeal mutilation with protuberant lips Am J Trop Med Hyg 59:49-52, 1998 Leprosy Rook p.3140, 1998, Sixth Edition Mycobacterium tuberculosis – cheilitis granulomatosa AD 141:1085-1091, 2005; AD 141:1080-1082, 2005; JAAD 23:444-450, 1990; AD 124:1705-1710, 1988 Orf Br Dent J 173:343-344, 1992 Paracoccidioidomycosis JAAD 31:S91-S102, 1994; Rev Bras Med 28:464, 1971 Parvovirus B19, including papular-purpuric ‘gloves and socks’ syndrome – swollen lips with painful erosions JAAD 41:793-796, 1999 Phaeoacremonium inflatipes – fungemia in child with aplastic anemia; swelling and necrosis of lips, periorbital edema, neck swelling Clin Inf Dis 40:1067-1068, 2005 Post-infectious lymphedema of lips Rhinoscleroma – upper lip swelling Cutis 40:101-103, 1987 Staphylococcus aureus Rook p.3140, 1998, Sixth Edition Syphilis – primary chancre – ulcerated papule with edema of lip Rook p.1244,3134, 1998, Sixth Edition; cheilitis granulomatosa AD 141:1085-1091, 2005; AD 141:1080-1082, 2005; JAAD 23:444-450, 1990; AD 124:1705-1710, 1988 Tinea facei (kerion) Rook p.3140, 1998, Sixth Edition Trichinosis Rook p.3140, 1998, Sixth Edition Vaccinia Br Dent J 143:57-59, 1977 Verruca vulgaris Yaws

Amyloidosis – primary systemic JAAD 50:815-817, 2004 Plasma cell orificial mucositis (plaque) AD 124:1871-1872, 1988; plasma cell cheilitis – with lip ulcer JAAD 30:789-780, 1994

Cheilitis granulomatosa – idiopathic, AD 141:1085-1091, 2005; AD 141:1080-1082, 2005; JAAD 23:444-450, 1990; AD 124:1705-1710, 1988 Crohn’s disease – orofacial granulomatosis JAAD 49:952-954, 2003; AD 135:439-442, 1999; Ped Derm 16:39-42, 1999; JAAD 36:697-704, 1997; cheilitis granulomatosa AD 141:1085-1091, 2005; AD 141:1080-1082, 2005; JAAD 23:444-450, 1990; AD 124:1705-1710, 1988 Erythema multiforme Medicine 68:133-140, 1989; JAAD 8:763-765, 1983; Stevens-Johnson syndrome Rook p.3133, 1998, Sixth Edition; Int J Derm 24:587-591, 1985

Granulation tissue Orofacial granulomatosis – facial edema with swelling of lips, cheeks, eyelids, forehead, mucosal tags, mucosal cobblestoning, gingivitis, oral aphthae; including Melkersson-Rosenthal syndrome, Miescher’s granulomatous cheilitis AD 124:1706, 1709, 1988; Crohn’s disease, sarcoid, allergy to food additives or cobalt BJD 143:1119-1121, 2000; Rook p.3139-3140, 1998, Sixth Edition; Quintessence International 28:265-269, 1997; AD 129:477-480, 1993 Pyoderma gangrenosum Br J Plast Surg 53:441-443, 2000; JAAD 18:559-568, 1988 Pyostomatitis vegetans Rook p.3142, 1998, Sixth Edition Sarcoid – granulomatous cheilitis AD 141:1085-1091, 2005; AD 141:1080-1082, 2005; Lupus 1:129-131, 1992; JAAD 23:444-450, 1990; AD 124:1705-1710, 1988; BJD 115:619-622, 1986; Rook p.2290, 1998, Sixth Edition; J Oral Surg 34:237-244, 1976

Congenital neutropenia Cyclic neutropenia Cystic fibrosis Fabry’s disease Ghatan p.76, 2002, Second Edition Fucosidosis type III Hunter’s syndrome – X-linked recessive; macrocheilia; reticulated 2-10 mm skin-colored papules over scapulae, chest, neck, arms; MPS type II; iduronate-2 sulfatase deficiency; lysosomal accumulation of heparin sulfate and dermatan sulfate; short stature, coarse facies, macroglossia, clear corneas (unlike Hurler’s syndrome), progressive neurodegeneration, communicating hydrocephalus, valvular and ischemic heart disease, lower respiratory tract infections, adenotonsillar hypertrophy, otitis media, obstructive sleep apnea, diarrhea, hepatosplenomegaly, skeletal deformities (dysostosis multiplex), widely spaced teeth, dolichocephaly, deafness, retinal degeneration, inguinal and umbilical hernias Ped Derm 21:679-681, 2004 Hurler’s syndrome Rook p.3140, 1998, Sixth Edition; Oral Surg 32:46-57, 1971 Hypothyroidism NEJM 283:101, 1970 Lesch-Nyhan syndrome – X-linked recessive; hypoxanthineguanine phosphoribosyltransferase deficiency; self-mutilation; biting of lower lip AD 94:194-195, 1966 Vitamin B1 and B2 deficiencies – red lips, angular cheilitis Ped Derm 16:95-102, 1999; JAAD 15:1263-1274, 1986

Actinic cheilitis J Derm Surg Oncol 7:289-295, 1981 Epstein-Barr virus associated lymphoproliferative lesions BJD 151:372-380, 2004 Granular cell schwannoma – linear plaque AD 121:1197-1202, 1985 Kaposi’s sarcoma Rook p.3130, 1998, Sixth Edition Leukemic macrocheilia – acute myelogenous leukemia BJD 151:1102, 2004; JAAD 14:353-358, 1986 Leukoplakia Lymphoma – T-cell lymphoma mimicking Crohn’s disease Oral Oncol 29B:225-230, 1993

Lymphomatoid granulomatosis (angiocentric lymphoma) – presenting as angioedema Postgrad Med J 68:366-368, 1992 Melanoma – desmoplastic melanoma of lip; induration and edema JAAD 47:863-868, 2002 Neurofibroma Rook p.3140, 1998, Sixth Edition Squamous cell carcinoma Verrucous carcinoma – oral florid papillomatosis of the lip BJD 151:727-729, 2004

Paraneoplastic pemphigus

Actinic prurigo BJD 144:194-196, 2001; JAAD 44:952-956, 2001; Ped Derm 17:432-435, 2000; JAAD 26:683-692, 1992; Oral Surg Oral Med Oral Pathol 65:327-332, 1988; Ped Derm 3:384-389, 1986; JAAD 5:183-190, 1981

Acanthosis nigricans JAAD 31:1-19, 1994 Acne vulgaris – cyst Atopic cheilitis Allergy 46:125-128, 1991; Dermatologica 177:360-364, 1988 Cheilitis glandularis (Volkmann’s cheilitis) (Puente’s disease) – inflammatory condition of the lower lip minor salivary glands; enlargement with a mucus ductal discharge, crusts and scale eversion, and hardening of the lip; deep-seated abscesses and fistulae BJD 148:362, 2003; Oral Surg Oral Med Oral Pathol 78:319-322, 1994; Oral Surg Oral Med Oral Pathol 62:654-656, 1986 J Derm Surg 1:372-375, 1985 Congenital sensory neuropathy with anhidrosis – lip erosions Ped Derm 11:231-236, 1994 Hydroa vacciniforme – cheilitis with lip ulcers Ped Derm 21:555-557, 2004 Lichen planus Premature dermatochalasis

Ackerman syndrome – taurodontism, pyramidal and fused molar roots, juvenile glaucoma, unusual upper lip Am J Phys Anthropol 38:681-694, 1973 Ascher’s syndrome – blepharochalasis with progressive enlargement of upper lip; increased thickness of eyelids AD 139:1075-1080, 2003; JAAD 29:650-651, 1993; Ped Derm 8:122-123, 1991; BJD 66:129-138, 1954; Klin Monatsbl Augenheilkd 65:86-97, 1920 Beare-Stevenson syndrome – cutis gyrata (furrowed skin), acanthosis nigricans, hypertelorism, swollen lips, swollen fingers, prominent eyes, ear anomalies, and umbilical herniation Skin and Allergy News p.37, Sept 2000 Char syndrome – short philtrum, patulous lips, ptosis, low-set pinnae Birth Defects 14 (6B):303-305, 1978 Coffin-Lowry syndrome J Med Genet 25:344-348, 1988 Coffin-Siris syndrome Rook p.3140, 1998, Sixth Edition Cowden’s syndrome Darier’s disease Double lip

Down’s syndrome Familial cold urticaria Br Dent J 175:417-418, 1993 Facial edema with eosinophilia Familial idiopathic macrocheilia Fountain syndrome – coarse face with progressive swelling of lips, mental retardation, sensorineural deafness, and musculoskeletal abnormalities J Med Genet 26:722-724, 1989 Fucosidosis Rook p.3140, 1998, Sixth Edition Gingival fibromatosis Hereditary angioneurotic edema Hughes syndrome – acromegaloid features and thickened oral mucosa; thickened fissured lips J Med Genet 22:119-125, 1985 Kawasaki’s disease – mucocutaneous lymph node syndrome – cheilitis Rook p.3135, 1998, Sixth Edition Keratosis-ichthyosis-deafness syndrome – bright red thickened lips; hyperkeratotic papules and plaques of face, scalp, trunk, extremities; exaggerated diaper dermatitis Ped Derm 13:105-113, 1996; BJD 122:689-697, 1990 Leprechaunism (Donohue syndrome) Ann Genet 30:221-227, 1987 Melkersson-Rosenthal syndrome (granulomatous cheilitis) Dermatol Clin 14:371-379, 1996; JAAD 21:1263-1270, 1989 Morateaux-Lamy syndrome (MPS VI) Helv Paediatr Acta 25:337-362, 1970 Multicentric reticulohistiocytosis Multiple mucosal neuroma syndrome IIB – thick fleshy everted lips with papules AD 139:1647-1652, 2003; JAAD 42:939-969, 2000; Ped Derm 8:124-128, 1991 Neurofibromatosis PHACES syndrome J Pediatr 122:379-384, 1993 Schei syndrome Setleis syndrome Smith-Magenis syndrome – upper lip eversion, brachycephaly, midface hypoplasia, prognathism, short broad hands with short fingers, clinodactyly of fifth fingers, fingertip pads, mental retardation Am J Med Genet 41:225-229, 1991 Sturge-Weber syndrome (encephalofacial angiomatosis) – facial port wine stain with homolateral leptomeningeal angiomatosis Oral Surg Oral Med Oral Pathol 22:490-497, 1966 Sweet’s syndrome JAAD 53:S135-138, 2005 Trichothiodystrophy syndromes – BIDS, IBIDS, PIBIDS – macrocheilia, poikiloderma, sparse or absent eyelashes and eyebrows, brittle hair, premature aging, sexual immaturity, ichthyosis, dysmyelination, bird-like facies, dental caries; trichothiodystrophy with ichthyosis, urologic malformations, hypercalciuria and mental and physical retardation Ped Derm 14:441-445, 1997; JAAD 44:891-920, 2001 X-linked anhidrotic ectodermal dysplasia Williams syndrome – premature laxity of skin, congenital heart disease (supravalvular aortic stenosis), baggy eyes, full cheeks, prominent lips, dental malocclusion, delayed motor skills, cocktail party personality J Pediatr 113:318-326, 1988 Winchester syndrome – lip hypertrophy, diffuse thickening, hyperpigmentation, hypertrichosis Ped Derm 21:154-159, 2004

Electrical burn Hematoma

Intubation – pressure necrosis of lip Anesthetis 24:136-137, 1975 Physical trauma Sunburn Surgery – prolonged intubation with pressure ulceration of mucosal surface of lower lip and accompanying edema

Arteriovenous malformation Elephantiasis nostras Oral Surg Oral Med Oral Pathol Oral Radiol Endod 84:297-300, 1997; J Allergy Clin Immunol 75:450-451, 1985; Angiology 22:448-455, 1971 Hemangioma (proliferating hemangioma; resolved hemangioma Rook p.563,3140, 1998, Sixth Edition Hematoma Kimura’s disease JAAD 38:143-175, 1998 Lymphangiectasia – macrocheilia South Med J 69:485-486, 1976 Lymphangioma Rook p.3140, 1998, Sixth Edition Lymphedema Pyogenic granuloma Sclerosing lymphangitis of the lip J Dermatol 17:127-129, 1990 Superior vena cava syndrome Vascular malformation Venous obstruction Venous lake Wegener’s granulomatosis Rook p.3079, 1998, Sixth Edition

Dermatomyositis – livedo reticularis and multiple ulcers J Eur Acad DV 11:48-50, 1998 Hypogammaglobulinemia, congenital JAAD 52:1009-1019, 2005 Lupus erythematosus – systemic lupus erythematosus BJD 135:355-362, 1996; livedo – of upper or lower extremities Rook p.2474, 1998, Sixth Edition; J Rheumatol 6:204-209, 1979; discoid lupus Rook p.964, 966, 2452, 1998, Sixth Edition; tumid lupus – livedoid telangiectasias JAAD 41:250-253, 1999; Rook p.2447, 1998, Sixth Edition; subacute cutaneous lupus erythematosus Med Clin North Am 73:1073-1090, 1989; JAAD 19:1957-1062, 1988; lupus vasculitis JAAD 48:311-340, 2003 Mixed connective tissue disease Rheumatoid arthritis Rook p.964, 2568, 1998, Sixth Edition; vasculitis JAAD 53:191-209, 2005; JAAD 48:311-340, 2003; BJD 147:905-913, 2002; intravascular or intralymphatic histiocytosis in rheumatoid arthritis; red livedoid plaques BJD 152:1385-1387, 2005; JAAD 50:585-590, 2004 Scleroderma AD 119:803-807, 1983 Sjögren’s syndrome JAAD 52:1009-1019, 2005 Still’s disease JAAD 52:1009-1019, 2005

Angioma serpiginosum Capillary nevi Congenital livedo reticularis Cutis marmorata telangiectatica congenita JAAD 20:1098-1104, 1989 Diffuse phlebectasia (Bockenheimer’s syndrome) Divry-van Bogaert syndrome (corticomeningeal angiomatosis) Klippel-Trenaunay-Weber syndrome Lupus erythematosus, neonatal Port wine stain with reticulate pattern

Brain injury JAAD 52:1009-1019, 2005 Encephalitis JAAD 52:1009-1019, 2005 Multiple sclerosis JAAD 52:1009-1019, 2005 Paralysis – stasis Parkinson’s disease JAAD 52:1009-1019, 2005 Poliomyelitis JAAD 52:1009-1019, 2005

Amantadine BJD 149:656-658, 2003; JAAD 125:417-422, 1989 Arsphenamine JAAD 52:1009-1019, 2005 Bismuth injection, intra-arterial Rook p.964, 1998, Sixth Edition Catecholamines JAAD 52:1009-1019, 2005 Coumarin – coumarin necrosis, coumarin purple toe syndrome Thromb Haemost 78:785-790, 1997 Ergotism, chronic Erythromycin/lovastatin interaction JAAD 52:1009-1019, 2005 Gemcitabine JAAD 52:1009-1019, 2005 GCSF – recombinant human GCSF; livedo reticularis, edema, with thrombotic and necrotizing panniculitis BJD 142:834-836, 2000 Heparin JAAD 52:1009-1019, 2005 Interferon-β-1b JAAD 37:553-558, 1997 Minocycline-induced p-ANCA+ cutaneous polyarteritis nodosa – vasculitis Eur J Dermatol 13:366-368, 2003; JAAD 48:311-340, 2003; JAAD 44:198-206, 2001 Pentazocine injection J R Soc Med 75:903-909, 1982 Quinidine – photosensitive purpuric livedo JAAD 12:332-336, 1985; Dermatologica 148:371-376, 1974; AD 108:100-101, 1973 Quinine Sonodor (diphenhydramine and pyrithyldione) JAAD 52:1009-1019, 2005

Arterial cannula Arterial embolization with hyaluronic acid – livedo reticularis of nose and face BJD 146:928-929, 2002 Carbon dioxide arteriography JAAD 52:1009-1019, 2005 Crack inhalation – acrocyanosis, necrotizing livedo reticularis (livedo racemosa), and muscle infarction Ann Intern Med 108:843, 1988

Intra-arterial injections Nitrogen – decompression sickness JAAD 52:1009-1019, 2005 Ventilator gas embolism JAAD 52:1009-1019, 2005

Bacterial endocarditis – acute or subacute Brucella JAAD 52:1009-1019, 2005 Coxiella burnetii JAAD 52:1009-1019, 2005 Endocarditis JAAD 52:1009-1019, 2005 Hepatitis C – symmetric polyarthritis with livedo reticularis JAAD 37:817-823, 1997 Infectious mononucleosis Meningococemia JAAD 52:1009-1019, 2005 Mycobacterium tuberculosis JAAD 52:1009-1019, 2005; Rook p.964, 1998, Sixth Edition Mycoplasma pneumoniae – cold agglutinins JAAD 52:1009-1019, 2005 Parvovirus B19 with myasthenia gravis-like syndrome AD 131:744-745, 1995 Pneumococcal sepsis Ghatan p.41, 2002, Second Edition Q fever (Coxiella burnetii ) JAAD 41:842-844, 1999 Rheumatic fever Rook p.2575, 1998, Sixth Edition Rickettsial diseases JAAD 52:1009-1019, 2005 Rubella – congenital rubella syndrome – seborrhea, cutis marmorata, hyperpigmentation JAAD 46:161-183, 2002 Septic emboli – with pseudoaneurysms due to Staphylococcus aureus following percutaneous transluminal coronary angioplasty; palpable purpura, petechiae, and livedo reticularis Cutis 66:447-452, 2000 Streptococcemia JAAD 52:1009-1019, 2005 Syphilis Rook p.964, 1998, Sixth Edition Typhus JAAD 52:1009-1019, 2005 Varicella – livedo reticularis as the only manifestation Tyring p.125, 2002 Viral syndromes JAAD 52:1009-1019, 2005

Amyloidosis JAAD 52:1009-1019, 2005 Intravascular or intralymphatic histiocytosis in rheumatoid arthritis – livedoid red patches JAAD 50:585-590, 2004

Fibromyalgia JAAD 52:1009-1019, 2005 Pancreatitis, chronic – livedo reticularis; ‘Walzel’s sign’ JAAD 39:1035-1036, 1998

Anti-thrombin III deficiency JAAD 52:1009-1019, 2005 Calciphylaxis (vascular calcification cutaneous necrosis syndrome) (chronic renal failure and hyperparathyroidism) JAAD 40:979-987, 1999; JAAD 33:53-58, 1995; JAAD 33:954-962, 1995;J Dermatol 28:27-31, 2001; Br J Plast Surg 53:253-255, 2000; J Cutan Med Surg 2:245-248, 1998; JAAD 33:954-962, 1995; AD 131:786, 1995; AD 127:225-230, 1991; Arch Int Med 136:1273-1280, 1976; acute reversible

renal failure; associated with hepatitis C infection JAAD 50:S125-128, 2004 Cardiac failure JAAD 52:1009-1019, 2005 Cold agglutininemia Rook p.964, 1998, Sixth Edition Cretinism – coarse facial features, lethargy, macroglossia, cold dry skin, livedo, umbilical hernia, poor muscle tone, coarse scalp hair, synophrys, no pubic or axillary hair at puberty Rook p.2708, 1998, Sixth Edition Cryofibrinogenemia Am J Med 116:332-337, 2004 Cryoglobulinemia JAAD 48:311-340, 2003; Rook p.964, 1998, Sixth Edition Cushing’s syndrome JAAD 52:1009-1019, 2005 Diabetes mellitus – peripheral neuropathy JAAD 52:1009-1019, 2005 Disseminated intravascular coagulation, including symmetric peripheral gangrene JAAD 52:1009-1019, 2005 Hemolytic uremic syndrome JAAD 52:1009-1019, 2005 Homocystinuria – cystathionine-beta synthase deficiency JAAD 40:279-281, 1999 Hypercalcemia JAAD 52:1009-1019, 2005 Hyperparathyroidism with vascular calcification Rook p.964, 1998, Sixth Edition Hyperviscosity Hypothyroidism JAAD 52:1009-1019, 2005 Pellagra JAAD 52:1009-1019, 2005 Pernicious anemia JAAD 52:1009-1019, 2005 Pregnancy – cutis marmorata of legs Ghatan p.297, 2002, Second Edition Primary oxalosis (primary hyperoxaluria) – type 1 – alanine glyoxalate aminotransferase (transaminase) deficiency; chromosome 2q36-37; type 2 (rare) – D-glyceric acid dehydrogenase deficiency JAAD 49:725-728, 2003; JAAD 46:S16-18, 2002; AD 137:957-962, 2001; JAAD 22:952-956, 1990; AD 131:821-823, 1995; AD 125:380-383, 1989; livedo reticularis, ulcers, and peripheral gangrene AD 136:1272-1274, 2000 Protein S and C deficiency JAAD 52:1009-1019, 2005 Renal disease, chronic – calciphylaxis Thrombocytopathy JAAD 52:1009-1019, 2005 Thrombotic thrombocytopenic purpura JAAD 52:1009-1019, 2005

Aortic tumors – aortic angiosarcoma with cutaneous metastases JAAD 43:930-933, 2000; primary aortic tumors Oncology 39:167-172, 1982 Atrial myxoma BJD 147:379-382, 2002; Cutis 62:275-280, 1998; JAAD 32:881-883, 1995; JAAD 25:110-111, 1991 Gamma heavy chain disease JAAD 23:988-991, 1990 Inflammatory breast carcinoma JAAD 52:1009-1019, 2005 Leukemia – chronic myelogenous leukemia with leukostasis; acral livedo AD 123:921-924, 1987; acute lymphocytic leukemia JAAD 52:1009-1019, 2005 Lymphomas – cutaneous T-cell lymphoma JAAD 52:1009-1019, 2005; intravascular large cell lymphoma JAAD 39:318-321, 1998 Multiple myeloma with paraproteins and crystaloglobulins JAAD 52:1009-1019, 2005 Livedo reticularis, primary JAAD 52:1009-1019, 2005

Nevus oligemicus AD 117:111-113, 1981 Pheochromocytoma JAAD 52:1009-1019, 2005 Polycythemia vera JAAD 26:264-265, 1992 Renal cell carcinoma JAAD 52:1009-1019, 2005 Essential thrombocythemia JAAD 24:59-63, 1991; Br J Haematol 36:553-564, 1977; AD 87:302-305, 1963 Waldenström’s macroglobulinemia AD 134:1127-1131, 1998; cryoglobulin-associated livedo reticularis JAAD 45:S202-206, 2001

Cutis marmorata – physiologic JAAD 52:1009-1019, 2005 Physiologic

Livedo reticularis, photosensitive AD 108:100-101, 1973

Lichen planus Rook p.966, 1998, Sixth Edition Livedo reticularis, primary JAAD 52:1009-1019, 2005 Nevus anemicus Psoriasis Cutis 39:429-432, 1987 Symmetrical lividity of the soles BJD 37:123-125, 1925

Adams-Oliver syndrome – aplasia cutis congenita, cutis marmorata telangiectatica congenital, transverse limb defects Ped Derm 22:206-209, 2005 Antiphospholipid antibody syndrome NEJM 346:752-763, 2002; Semin Arthritis Rheum 31:127-132, 2001; JAAD 36:149-168, 1997; JAAD 36:970-982, 1997; BJD 120:419-429, 1989; IgG-1 antibodies to β2-glycoprotein 1 Am J Med 101:472-281, 1996 Carcinoid syndrome JAAD 52:1009-1019, 2005 Cardio-facio-cutaneous syndrome Cornelia de Lange (Brachmann-de Lange) syndrome – generalized hypertrichosis, confluent eyebrows, low hairline, hairy forehead and ears, hair whorls of trunk, cutis marmorata, single palmar crease, physical and mental retardation JAAD 52:1009-1019, 2005; Am J Med Genet 47:959-964, 1993; marbling around the eyes and nose Corticomeningeal angiomatosis Eur Neurol 9:202-215, 1973 Down’s syndrome JAAD 52:1009-1019, 2005 Macrocephaly cutis marmorata syndrome – hemangioma of philtrum of upper lip, syndactyly of toes, hyperelastic skin capillary and cavernous hemangiomas Clin Dysmorphol 6:291-302, 1997 Neurofibromatosis – vasculopathy JAAD 51:656-659, 2004; neurofibromatosis type I – plexiform neurofibroma with pressure-induced arterial occlusion and livedo racemosa JAAD 50:S107-109, 2004 Nicolau syndrome – following intramuscular injection (of bismuth, diclofenac, ibuprofen), intra-arterial, acute ischemia, severe pain, livedo, necrosis of skin and muscle, dendritic extension, ulcer, necrotic eschar, atrophic scar BJD 150:385-387, 2004; J Eur Acad Dermatol 15:585-588, 2001; Ped Derm 12:187-190, 1995; Ann Intern Med 117:1058, 1992

Phakomatosis cesiomarmorata – Mongolian spot and cutis marmorata telangiectatica congenita AD 141:385-388, 2005 POEMS syndrome JAAD 37:887-920, 1997; JAAD 40:808-812, 1999; JAAD 19:979-982, 1988 Reflex sympathetic dystrophy JAAD 52:1009-1019, 2005 Relapsing polychondritis Clin Exp Rheumatol 20:89-91, 2002 Riley-Day syndrome (familial dysautonomia) – mottling Rook p.2734, 1998, Sixth Edition Rothmund-Thomson syndrome Sharp’s syndrome JAAD 52:1009-1019, 2005 Sneddon’s syndrome – livedo reticularis associated with arterial disease of cerebral, coronary, renal, and peripheral vessels; hypertension and neurologic signs and symptoms; cerebrovascular episodes; antiphospholipid antibodies and lupus anticoagulant often but not invariably present Cutis 67:211-214, 2001; BJD 142:374-376, 2000; Sem Derm 14:166-172, 1995; JAAD 22:633-639, 1990; Int J Dermatol 29:45-49, 1990; BJD 77:180-185, 1965; livedo racemosa generalisata JAAD 22:633-9, 1990; cutaneous thrombosis, cerebrovascular thrombosis, and lupus anticoagulant Int J Dermatol 29:45-49, 1990 Trisomy 18 JAAD 52:1009-1019, 2005

Acrodynia (pink disease) – reticulate erythema of hands; mercury poisoning

Cold injury – cutis marmorata Compressed air illness (decompression sickness) (Caisson’s disease) Rook p.964, 1998, Sixth Edition Erythema ab igne Rook p.937,967, 1998, Sixth Edition Frostbite Infrared radiation – erythema ab igne Intra-arterial cannula Intravenous drug abuse – intra-arterial buprenorphine tablets injected within brachial artery BJD 150:1-10, 2004 Midline catheter insertion JAAD 52:1009-1019, 2005

Arteriosclerosis Rook p.964,2229, 1998, Sixth Edition Atrophie blanche – livedo with summer (winter) ulcerations JAAD 8:792-798, 1983; AD 119:963-969, 1983 Cholesterol emboli BJD 146:1107-1108, 2002; BJD 146:511-517, 2002; BJD 146:511-517, 2002; Rook p.2229, 1998, Sixth Edition; Medicine 74:350-358, 1995; Angiology 38:769-784, 1987; AD 112:1194-1198, 1986; JAAD 13:235-242, 1985 Churg-Strauss disease Medicine 78:26-37, 1999; JAAD 37:199-203, 1997; BJD 127:199-204, 1992 Coarctation of the aorta – cutaneous mottling of arm BJD 148:1066-1068, 2003 Congestive heart failure – stasis Down’s syndrome Rook p.373, 1998, Sixth Edition Factor XII deficiency – livedo with ulceration BJD 143:897-899, 2000 Fat emboli JAAD 52:1009-1019, 2005 Hypertensive ulcer (Martorell’s ulcer) – very painful ulcer of lower lateral leg with livedo at edges Phlebology 3:139-142, 1988

Intramural thrombus – embolus Klippel-Trenaunay-Weber syndrome Livedo racemosa Livedo reticularis – acquired idiopathic Livedo reticularis with winter ulceration Malignant angioendotheliomatosis (angiocentic lymphoma) – scalp; livedoid red plaque of thigh with woody induration Rook p.2396, 1998, Sixth Edition; JAAD 18:407-412, 1988 Moyamoya disease – narrowing or occlusion of supraclinoid portion of the internal carotid artery and stems of anterior and middle cerebral arteries Ped Derm 20:124-127, 2003 Neurologic syndromes – cerebral vascular accidents, transient ischemic attacks Parkes-Weber syndrome Peripheral vascular disease

Vessel wall disease – arteriosclerotic, hyperparathyroidism, arteritis (PAN, rheumatoid arthritis, LE, dermatomyositis, lymphoma, syphilis, tuberculosis, pancreatitis) JAAD18:1003-1019, 1988

Port wine stain (nevus flammeus) with reticulate pattern Primary pulmonary hypertension Raynaud’s disease and phenomenon Sturge-Weber syndrome Symmetric peripheral gangrene Thromboangiitis obliterans (Buerger’s disease) Rook p.2233, 1998, Sixth Edition; Am J Med Sci 136:567-580, 1908 Tumor emboli Vascular malformation with overlying slow flow Vasculitis (adopted from Current Prob Derm, Vol V, No.2, 1993)

Small vessel vasculitis Circulating immune complexes Collagen vascular disease – SLE, rheumatoid arthritis, dermatomyositis, Sjögren’s syndrome, scleroderma Cutis 39:429-432, 1987 Drug-induced vasculitis Erythema elevatum diutinum Familial Mediterranean fever Henoch-Schönlein purpura; HSP of adults AD 125:53-56, 1989 Livedoid vasculitis – livedo with summer ulcerations JAAD 51:574-579, 2004 Mixed cryoglobulinemia Organ transplant vasculitis Paraneoplastic vasculitis Serum sickness Transplacental transient vasculitis JAAD 52:1009-1019, 2005 Vasculitis of C2 deficiency Vasculitis associated with cystic fibrosis Vasculitis associated with subacute bacterial endocarditis Small vessel lymphocytic vasculitis with winter or summer ulcerations

Small and medium-sized vessel vasculitis Churg-Strauss granulomatosis JAAD 47:209-216, 2002 Dermatomyositis Lupus erythematosus Lymphomatoid granulomatosis Lymphomas Microscopic polyangiitis JAAD 52:1009-1019, 2005 Necrotizing sarcoid granulomatosis Nodular vasculitis JAAD 52:1009-1019, 2005 Pancreatitis Polyarteritis nodosa (PAN) – nodules along the course of superficial arteries around knee, anterior lower leg and dorsum of foot JAAD 53:724-728; JAAD 48:311-340,

2003; JAAD 31:561-566, 1994; Ann Rheum Dis 54:134-136, 1995; Ann Intern Med 89:66-676, 1978; infantile systemic – red patch heralding cutaneous infarction J Pediatr 120:206-209, 1992 Polyarteritis nodosa, cutaneous (livedo with nodules) – starburst livedo; painful or asymptomatic red or skin colored multiple nodules with livedo reticularis of feet, legs, forearms face, scalp, shoulders, trunk JAAD 52:1009-1019, 2005; BJD 146:694-699, 2002; Ped Derm 15:103-107, 1998; JAAD 31:561-566,1994; cutaneous associated with Crohn’s disease Dis Colon Rectum 23:258-262, 1980 Rheumatoid arteritis Syphilis Tuberculosis Vasculitis of collagen vascular diseases Wegener’s granulomatosis

Large vessel vasculitis Giant cell arteritis Takayasu’s arteritis

Infectious vasculitis Bacterial, including meningococcemia, Vibrio vulnificus, etc. Fungal – including cryptococcosis, aspergillosis Mycobacterial Mycoplasmal Protozoal Rickettsial Septic emboli JAAD 52:1009-1019, 2005 Spirochetal – syphilis, Borrelial Viral – cytomegalovirus AD 116:1174-1176, 1980

Thrombotic vasculitis Antiphospholipid antibodies Anti-thrombin III deficiency Caisson’s disease – intravascular occlusion Cardiac failure – intravascular obstruction Cryofibrinogenemia Cryoglobulinemia Emboli – intravascular occlusion Hyperviscosity – intravascular occlusion Hypercoagulable states (DIC) Macroglobulinemia Paralysis – intravascular obstruction Protein C or S deficiency Sneddon’s syndrome Stasis – intravascular obstruction Thrombocytosis

Venous gangrene Venous thrombosis, deep JAAD 52:1009-1019, 2005

Lupus erythematosus Rheumatoid arthritis

Congenital lipomatosis – adipose tissue malformation Rook p.550, 1998, Sixth Edition Double digit Duplication of the thumb JAAD 39:114-115, 1998

Monstrous congenital macrodactyly with syndactyly of the foot Acta Orthop Scand 69:201-202, 1998 Syndactyly Caputo p.172, 2000

Osteoarthritis

Foreign body granuloma

Brodie abscess – chronic abscess of bone surrounded by dense fibrous connective tissue and sclerotic bone Eikenella corrodens – cellulitis Clin Infect Dis 33:54-61, 2001; felon with fingertip necrosis Diabetes Care 19:1011-1013, 1996 Erysipelas Erysipeloid Felon Leprosy Mycobacterium tuberculosis – tuberculous dactylitis Seal finger (speck finger) – Mycoplasma species (M. phocacerebrale and M. phocarhinis) Clin Infect Dis 27:1168-1170, 1998 Syphilis, congenital Tenosynovitis – acute suppurative tenosynovitis Warts

Amyloidosis Sarcoidosis Xanthoma disseminatum AD 121:1313-1317, 1985

Chronic granulomatous paronychia – pseudomegadactyly Dermatologica 169:86-87, 1984 Osteoperiostitis

Acromegaly Adrenal tumor with hemihypertrophy JAAD 33:154-155, 1995 Clubbing (Hippocratic fingers) (acropachy) JAAD 52:1020-1028, 2005; Rook p.422-423, 1998, Sixth Edition; Ghatan p.79, 2002, Second Edition; JAMA 286:341-347, 2001

Amyloid, liver abscess Aortic aneurysm Arsenic Asbestosis JAAD 52:1020-1028, 2005 Asymmetric Unidigital

Enchondroma Rook p.2847, 1998, Sixth Edition Epidermoid cyst Felon

Gout Median nerve injury Osteoid osteoma Rook p.2847, 1998, Sixth Edition Sarcoid Trauma

Unilateral Aneurysm – left innominate artery Axillary Pancoast tumors Brachial arteriovenous aneurysm Patent ductus arteriosus with pulmonary hypertension Prolonged hemiplegia

Bronchogenic carcinoma Bronchiectasis, cysts Carcinoma of the nasopharynx, colon, esophagus, stomach Ghatan p.79, 2002, Second Edition Celiac sprue JAAD 52:1020-1028, 2005 CINCA syndrome JAAD 52:1020-1028, 2005 Cirrhosis Crohn’s disease Congestive heart failure Cyanotic congenital heart disease JAAD 52:1020-1028, 2005 Cystic fibrosis JAAD 52:1020-1028, 2005 Diarrhea, chronic Down’s syndrome Familial Hemochromatosis Hereditary – autosomal dominant Hereditary mucoepithelial dysplasia HIV disease JAAD 52:1020-1028, 2005 Hyperparathyroidism Hypertrophic osteoarthropathy Hypersensitivity pneumonitis JAAD 52:1020-1028, 2005 Hypervitaminosis A Idiopathic Idiopathic pulmonary fibrosis JAAD 52:1020-1028, 2005 Infective endocarditis JAAD 52:1020-1028, 2005 Inflammatory bowel disease JAAD 52:1020-1028, 2005 Juvenile polyposis coli JAAD 52:1020-1028, 2005 Laxative abuse SMJ 76:1071, 1983; Lancet 2:947, 1978 Liver disease JAAD 52:1020-1028, 2005 Lung abscess, empyema Lupus erythematosus BJD 89:533-535, 1973 Lymphoma JAAD 52:1020-1028, 2005 Malabsorption Malaria Malnutrition Ghatan p.79, 2002, Second Edition Marfan’s syndrome – pseudoclubbing Myxedema, Graves’ disease (thyroid acropachy) Med Clin North Am 52:393-395, 1968 Marie-Bamberger syndrome Mesothelioma JAAD 52:1020-1028, 2005 Nasopharyngeal carcinoma JAAD 52:1020-1028, 2005 Parasitic bacillary dysentery JAAD 52:1020-1028, 2005 Phosphorus toxicity Pleural tumors JAAD 52:1020-1028, 2005 POEMS syndrome JAAD 52:1020-1028, 2005 Polycythemia Psoriatic onycho-pachydermoperiostitis BJD 143:901-902, 2000; Rev Rheum Mal Osteoartic 56:579-582, 1989 Pulmonary arteriovenous malformation JAAD 52:1020-1028, 2005 Pycnodysostosis – pseudoclubbing (acro-osteolysis) Br Med J iii:712-714, 1967 Sarcoidosis JAAD 52:1020-1028, 2005 Sprue Subacute bacterial endocarditis Syringomyelia Systemic lupus erythematosus Thymoma, malignant

Thyroid disease JAAD 52:1020-1028, 2005 Tuberculosis JAAD 52:1020-1028, 2005 Ulcerative colitis Venous stasis JAAD 52:1020-1028, 2005 X-linked reticulate pigmentary disorder with systemic manifestations (familial cutaneous amyloidosis) (Partington syndrome II) – X-linked; rare; Xp21-22; boys with generalized reticulated muddy brown pigmentation (dyschromatosis) with hypopigmented corneal dystrophy (dyskeratosis), coarse unruly hair, unswept eyebrows, silvery hair, hypohidrosis, recurrent pneumonia with chronic obstructive disease, clubbing; failure to thrive, female carriers with linear macular nevoid Blascko-esque hyperpigmentation Ped Derm 22:122-126, 2005; Semin Cut Med Surg 16:72-80, 1997; Am J Med Gen 10:65:1981

Gout Hyperthyroidism – thyroid acropachy JAAD 26:885-902, 1992 Mucopolysaccharidoses – Sly syndrome, Hurler’s syndrome Multiple sulfatase deficiency – broad thumbs, ichthyosis, gargoylism Ped Derm 18:388-392, 2001 Pulmonary osteoarthropathy Tuberous xanthomas JAAD 13:1-30, 1986

Aggressive digital papillary adenoma (adenocarcinoma) Cutis 69:179-182, 2002; AD 120:1612, 1984 Chondrosarcoma Connective tissue nevi JAAD 33:154-155, 1995 Epidermal nevus JAAD 33:154-155, 1995 Epidermoid cyst Rook p.2847, 1998, Sixth Edition; intraosseous epidermoid cyst BJD 145:366-368, 2001 Epithelioid sarcoma JAAD 26:302-305, 1992 Exostosis JAAD 19:132, 1988 Fibrolipoma Fibromatosis – localized digital fibromatosis Acta DV 74:386-387, 1994; Clin Exp Dermatol 16:121-123, 1991 Fibro-osseous pseudotumor of the digits BJD 144:1274-1275, 2001 Fibrosarcoma Fibrous hamartoma Ped Derm 17:270-276, 2000 Garlic clove tumor – acquired periungual fibrokeratoma Cutis 46:118-124, 1990 Giant cell tumor of the tendon sheath BJD 146:125-128, 2002 Glomus tumor Growth promoting neoplasm – adrenocortical tumor, testicular tumor, Wilms tumor Ped Derm 17:270-276, 2000 Infantile desmoid-type fibromatosis Ped Derm 12:149-151, 1995 Intraosseous chondroid syringoma JAAD 30:374-378, 1994 Kaposi’s sarcoma – epidemic; endemic African Kaposi’s sarcoma JAAD 22:1237-1250, 1990 Lipofibromatous hamartoma J Hand Surg 13:67-75, 1988; lipofibromatous hamartoma of the median nerve Ped Derm 15:378-380, 1998; congenital lipofibromatosis Foot Ankle 12:40-46, 1991; hyperostotic macrodactyly and lipofibromatous hamartoma of the median nerve Chir Main 18:261-271, 1999; fibrolipomatous hamartoma of the nerve (nerve territory-oriented macrodactyly) (macrodystrophia lipomatosa) Skeletal Radiol 24:296-297, 1995

Lipomas BJD 146:125-128, 2002; J Foot Ankle Surg 38:223-226, 1999; J Bone Surg Am 70:128-130, 1998; subungual lipoma BJD 149:418, 2003 Macrodystrophia lipomatosa (hamartomatous macrodactyly) Ped Derm 17:270-276, 2000 Melanoma, subungual Caputo p.94, 2000 Metastases – especially bronchogenic carcinoma JAAD 31:259-263, 1994 Myxoid cyst BJD 146:125-128, 2002 Neural fibrolipoma (neurofibrolipomatous hamartoma) JAAD 53:528-529, 2005; Ped Derm 17:270-276, 2000; AD 135:707-712, 1999 Neurinoma Neurofibroma, including plexiform neurofibroma J Pediatr Orthop 6:489-492, 1986; subungual BJD 146:125-128, 2002 Onychoblastoma (hamartoma of the nail unit) BJD 152:1077-1078, 2005 Osteochondroma Osteocystoma Osteoid osteoma J Hand Surg 12B:387-390, 1987 Osteogenic sarcoma Osteophytes Foot Ankle Int 17:283-285, 1996 Pachydermodactyly – fusiform swelling of fingers AD 141:1035-1040, 2005 Perineuroma (subungual) BJD 146:125-128, 2002 Plexiform schwannoma (neurilemmoma) J Hand Surg 21:707-710, 1996 Recurrent digital fibrous tumors of childhood

Digital gigantism (double digit) JAAD 33:154-155, 1995 Epidermolysis bullosa, junctional – letalis; atrophicans generalisata gravis, Herlitz type – extensive blistering and erosions at birth; bulbous finger tips with crusting and erosions Rook p.1828-1829, 1998, Sixth Edition Erythema elevatum diutinum Caputo p.29, 2000 Familial macrodactyly Arch Ped Adolescent Med 148:1309-1310, 1994; mother to daughter J Pods 5:650, 1935; siblings Arch Neurol and Psych 14:824, 1925 Hyperostotic macrodactyly J Hand Surg 13:544-548, 1988 Idiopathic macronychia and macrodactyly Ped Derm 17:270-276, 2000 Macrodactylia simplex congenita – static type, digit enlarged at birth, does not grow – usually a form of lipomatosis Ped Derm 17:270-276, 2000; S Ar Med J 70:755-758, 1986 Progressive macrodactyly J Foot Ankle Surg 38:143-146, 1999 Psoriatic onychopachydermoperiostitis BJD 153:858-859, 2005 Racket nails Segmental fibrous dysplasia Genet Couns 10:373-376, 1999 Tarsomegaly

Aase-Smith syndrome II – triphalangeal thumbs J Pediatr 74:471-474, 1969 Acrocallosal syndrome – broad halluces Helv Paediatr Acta 34:141-146, 1979

Acrofacial dysostosis – triphalangeal thumbs Am J Med Genet 14:225-229, 1983 ADULT (acro-dermato-ungual-lacrimal-tooth) syndrome – closely resemble EEC syndrome; hypodontia, ectrodactyly, obstruction of lacrimal ducts, onychodysplasia, freckling Am J Med Genet 45:642-648, 1993 Apert syndrome – acrocephalopolysyndactyly; broad halluces Birth Defects 11:137-189, 1975 Auro-digital-anal syndrome – broad thumbs Bannayan-Zonana-Ruvalcaba syndrome – macrodactyly, subcutaneous lipomatosis, megalencephaly Acta Pathol Jpn 36:269-277, 1986; Ped Derm 17:270-276, 2000 Carpenter syndrome (acrocephalsyndactyly type II) – broad halluces Am J Dis Child 124:716-718, 1972 Craniofrontonasal dysplasia – broad halluces Birth Defects 15:85-89, 1979 del (4p) syndrome (Wolf-Hirschhorn syndrome) – broad halluces Am J Med Genet 21:351-358, 1985 del (7p) syndrome – broad halluces Hum Genet 35:117-123, 1976 Dysplasia epiphysialis hemimelica J Hand Surg (Br) 10:249-250, 1985 Ectrodactyly-ectodermal dysplasia cleft lip/palate (lobster claw deformity) (EEC syndrome) – peg-shaped teeth Clin Dysmorphol 5:115-127, 1996; JAAD 29:347-350, 1993 Facio-digito-genital syndrome – syndactyly Familial mandibuloacral dysplasia (craniomandibular dermatodysostosis) – atrophy of skin over hands and feet with club shaped terminal phalanges and acro-osteolysis, mandibular dysplasia, delayed cranial suture closure, short stature, prominent eyes and sharp nose BJD 105:719-723, 1981; Birth Defects x:99-105, 1974 Femoral hypoplasia-unusual facies syndrome – broad halluces Syndromes of the Head and Neck, p.31, 1990 FG syndrome – broad halluces Am J Med Genet 19:379-382, 1984 Frontonasal malformation – broad halluces Clin Genet 10:214-217, 1976 Gardner’s syndrome – autosomal dominant; osteomas of skull, epidermoid cysts, and fibromas before age 10; polyposis of colon Goltz’s syndrome Gorham’s syndrome Greig cephalopolysyndactyly syndrome (frontodigital syndrome) – broad halluces Clin Genet 24:257-265, 1983 Jackson-Weiss syndrome – broad halluces J Pediatr 88:963-968, 1976 Juvenile hyaline fibromatosis JAAD 16:881-883, 1987 Kasabach-Meritt syndrome JAAD 29:117-119, 1993 Keipert syndrome (broad terminal phalanges and facial abnormalities) Aust Paediatr J 9:10-13, 1973 Klippel-Trenaunay-Weber syndrome Cutis 60:127-132, 1997; J Bone Joint Surg Am 73:1537-1546, 1991 LADD syndrome – triphalangeal thumbs Eur J Pediatr 146:536-537, 1987 Larsen syndrome Syndromes of the Head and Neck, p.722-724, 1990 Macrodystrophia lipomatosa progressiva – dynamic type; overgrowth of fatty tissue occurs and digits enlarge

dysproportionately Arch Orthop Traum Surg 110:220-221, 1991; A J Roentgenol 128:101-105, 1977 Maffucci’s syndrome (enchondromatosis) Ped Derm 17:270-276, 2000 Maghazaji syndrome Dev Med Child Neurol 25:798-800, 1983 Multiple exostoses syndrome JAAD 25:333-335, 1991 Multiple symmetric lipomatosis of the hands Clin Exp Dermatol 14:58-59, 1989 Munchmeyer’s syndrome – broad thumb Neurofibromatosis Am J Pathol 261:1059-1083, 1950; Ped Derm 17:270-276, 2000; JAAD 43:1136-1137, 2000 Nevus comedonicus syndrome Ped Derm 15:304-306, 1998 Oculo-dento-digital dysplasia Ollier’s disease – multiple enchondromata Ped Derm 12:55-58, 1995; Ped Derm 17:270-276, 2000 Oral-facial-digital syndromes – types 1, 2 – syndactyly Oto-palato-digital syndrome – broad thumb Birth Defects 5:43-44, 1969; Am J Dis Child 113:214-221, 1967 Pachydermodactyly JAAD 27:303-305, 1992; distal pachydermodactyly (acquired digital fibrosis) JAAD 38:359-362, 1998 Pachydermoperiostosis – primary (Touraine-Solente-Gole syndrome) – autosomal dominant – folds and furrows of forehead and cheeks, heavy thick eyelids, cutis verticis gyrata; thick skin of hands and feet; palmoplantar hyperhidrosis; enlarged hands JAAD 31:947-953, 1994; Medicine 70:208-214, 1991; AD 124:1831-1834, 1988; secondary – pulmonary disease, lung cancer, carcinoma of stomach, esophagus, thymus Pfeiffer syndrome Birth Defects 11:137-189, 1975 Proteus syndrome – macrodactyly, hemihypertrophy, lipomas, lymphangiomas, epidermal nevi, decreased subcutaneous fat, superficial venous prominence, palmar or plantar cerebriform connective tissue nevi, cutis marmorata, exostoses, macrocephaly, asymmetric growth AD 140:947-953, 2004; AD 137:219-224, 2001; JAAD 25:377-383, 1991; Ped Derm 17:270-276, 2000; Ped Derm 11:222-226, 1994 Robinson’s syndrome – nail dystrophy-deafness syndrome – polysyndactyly Rubinstein-Taybi syndrome – broad thumb JAAD 46:161-183, 2002; JAAD 46:159, 2002; Ped Derm 19:177-179, 2002; Ped Derm 11:21-25, 1994; mental deficiency, small head, broad thumbs and great toes, beaked nose, malformed low-set ears, capillary nevus of forehead, hypertrichosis of back and eyebrows, keloids, cardiac defects Cutis 57:346-348, 1996; Am J Dis Child 105:588-608, 1963 Sly syndrome Saethre-Chotzen syndrome – broad halluces J Pediatr 76:928-930, 1970 Temtamy syndrome J Ped 89:924-927 Townes-Brocks syndrome – triphalangeal thumbs Dysmorphol Clin Genet 2:104-108, 1988 Trichorhinophalangeal syndrome Trisomy 21 Tuberous sclerosis – Koenen’s tumors, periosteal hyperplasia, fibrous plaques of hand and fingers Ped Derm 18:364-365, 2001; Ped Derm 17:463-465, 2000; Skeletal Radiol 28:116-118, 1999; J R Soc Med 87:419-420, 1994

Weaver syndrome J Med Genet 17:174-178, 1980 Zunich syndrome – broad second toes Ped Derm 13:363-371, 1996

Physical trauma

Aneurysm – cirsoid aneurysm (arteriovenous fistula) – blue non-pulsatile nodules of nail fold with macrodactyly BJD 115:361-366, 1986 Angiodysplasia, localized Ann Med Interne (Paris) 140:237-239, 1989 Blue rubber bleb nevus syndrome AD 129:1505-1509, 1993 Hemangioma, congenital, subungual – pseudoclubbing JAAD 53:S123-126, 2005 Lymphangiosarcoma Lymphatic malformation Lymphedema, congenital (Milroy’s disease) or acquired – chronic Ped Derm 17:270-276, 2000 Vascular hamartoma Ped Derm 17:270-276, 2000 Vascular malformation Textbook of Neonatal Dermatology, p.326, 2001; Klippel-Trenaunay-Weber Ped Derm 17:270-276, 2000

MARFANOID HABITUS Alpha-2-deficient collagen disease Klin Wschr 52:906-912, 1974 Beals-Hecht syndrome (congenital contractural arachnodactyly) – autosomal dominant; crumpled ears, arachnodactyly with congenital joint contractures, confused with Marfan’s syndrome but no cardiovascular abnormalities Am J Med Genet 25:763-773, 1986; J Bone Joint Surg 53:987-993, 1971 Camurati-Engelmann disease type II – progressive diaphyseal dysplasia with striation of bones; waddling gait, muscle weakness, intense leg pain J Med Genet 107:5-11, 2002 Cataract, microcornea, dental anomalies Ophthalmology 86 (10):1764-1793, 1979 Cerebro-oculo-renal syndrome of Bhaskar Ophthalmology 86 (10):1764-1793, 1979 Cohen syndrome – mental retardation, congenital hypotonia, microcephaly, pleasant affect, micrognathia, open mouth with prominent incisors Congenital contractural acachnodactyly – joint contractures (elbows and knees), crumpled ears Cotlier-Reinglass syndrome Ophthalmology 86 (10):1764-1793, 1979 Cowden’s syndrome Craniostenosis Cutis laxa – congenital cutis laxa type V with Marfanoid habitus Ann DV 117:823-824, 1990 Cytoplasmic body and mitochodrial DNA deletion – marfanoid habitus and perceptive hearing loss J Neurol Sci 99:291-300, 1990 Diverticulosis, hernia, retinal detachment Ophthalmology 86 (10):1764-1793, 1979 Ehlers-Danlos syndrome type VIa JID 103:47S-52S, 1994 Familial Marfan habitus Am J Med Genet 62:417-426, 1996

Familial mitral valve prolapse syndrome Am J Med Genet 62:417-426, 1996 Familial myopathy with marfanoid features and multicores Aust N Z J Med 14:495-499, 1984 Goodman campylodactyly syndrome B Ophthalmology 86 (10):1764-1793, 1979 Homocystinuria – cystathionine-β synthase deficiency – marfanoid habitus, malar rash, larger facial pores, livedo reticularis, tissue paper scars, sparse fine hair, superficial thrombophlebitis JAAD 46:161-183, 2002; JAAD 40:279-281, 1999 Klinefelter syndrome Ophthalmology 86 (10):1764-1793, 1979 Klippel-Trenaunay syndrome Loeys-Dietz syndrome Nature Genet 37:275-281, 2005 Lujan-Fryns syndrome – X-linked; hypernasal voice, large head and long narrow face; mental retardation with marfanoid habitus Am J Med Genet 119A:363-366, 2003 Marfan’s syndrome – long extremities, arachnodactyly, skeletal, ocular, cardiovascular defects Rook p.2030-2031, 1998, Sixth Edition; Am J Med Genet 62:417-426, 1996; Int J Derm 28:291-299, 1989 Marfan’s syndrome with neurogenic muscle atrophy Kurume Med J 38:275-279, 1991 Marfanoid body habitus with crystalline lens subluxation, acute exophthalmos with intraocular hemorrhage and coma Medicina (Buenos Aires) 52:563-569, 1992 Marfanoid body habitus with acquired tracheobronchomegaly Chest 98:491-492, 1990 Marfanoid body habitus – with excessive hyperextensibility (Ehlers-Danlos syndrome type VIB) Am J Med Genet 36:269-272, 1990; Nippon Naika Gakkai Zasshi 77:499-505, 1988 Marfanoid body habitus with situs inversus Am J Med Genet 127A:310-312, 2004 Marfanoid features, visceral diverticula, diaphragmatic eventration Arch Dis Child 75:247-248, 1996 Marfanoid hypermobility syndrome – microcephaly, mental retardation, focal glomerulonephritis Clin Dysmorphol 1:111-113, 1992; Ann Intern Med 71:349-352, 1969 Marfanoid phenotype, congenital contractures with ocular and cardiovascular anomalies, cerebral white matter hypoplasia, and spinal axonopathy Eur J Pediatr 143:228-231, 1985 MASS phentotype – myopia, mitral valve prolapse, mild aortic dilatation, skin striae, skeletal involvement Am J Med Genet 62:417-426, 1996 Megaduodenum megacystis Ophthalmology 86 (10):1764-1793, 1979; Arch Surg 96:549-553, 1968 Mental retardation, X-linked, Snyder-Robinson type Clin Pediatr 8:669-674, 1969 Methylmalonic aciduria and homocystinuria, cb1C type Biochem Med 4:500-515, 1970 Mirhosseini syndrome Ophthalmology 86 (10):1764-1793, 1979 Multifocal Langerhans cell granulomatosis and diabetes insipidus with marfanoid habitus J Assoc Physicians India 36:665-667, 1988 Multiple endocrine neoplasia, type I – marfanoid habitus, optic atrophy MEN IIA – Sipple syndrome Multiple mucosal neuroma syndrome (MEN IIB) (Gorlin’s syndrome) – skin-colored papules and nodules of lips, tongue, oral mucosa AD 139:1647-1652, 2003; JAAD 36:296-300, 1997; Am J Med 31:163-166, 1961

MEN type III – Froboese’ syndrome Myotonic dystrophy Ophthalmology 86 (10):1764-1793, 1979 Nemaline myopathy Ophthalmology 86 (10):1764-1793, 1979 Nerve deafness, eye anomalies, and Marfanoid habitus – autosomal dominant Birth Defects: Original Article Series. 07 (4):137-139, 1971 Nevoid basal cell carcinoma syndrome JAAD 11:98-104, 1984 Pachydermoperiostosis, cryptorchidism, and marfanoid appearance Arch Belg Dermatol Syphiligr 23 (1):121-135, 1967 Peculiar facies and marfanoid habitus with psychomotor retardation Clin Genet 25:187-190, 1984 Shprintzen-Goldberg syndrome – marfanoid features and craniostenosis (craniosynostosis), mental retardation Am J Med Genet 76:202-212, 1998; J Craniofac Genet Dev Biol 2:65-74, 1982; without mental retardation Clin Dysmorphol 2:220-224, 1993 Sickle cell anemia Stickler syndrome (hereditary arthro-ophthalmopathy) – autosomal dominant; flat midface, cleft palate, myopia with retinal detachment, cataracts, hearing loss, arthropathy J Med Genet 36:353, 359, 1999; Birth Defects 11:77-103, 1975 Tall people Rook p.2031, 1998, Sixth Edition Transforming growth factor-beta receptor type II (TGFBR2) Nature Genet 36:855-860, 2004 Wagner-Stickler syndrome (dystrophia vitreoretinalis hereditaria) – hereditary arthro-ophthalmopathy 47,XYY syndrome Am J Dis Child 124:266-277, 1972

Lupus erythematosus, including tumid lupus JAAD 41:250-253, 1999; Am J Dermatopathol 21:356-360, 1999 Rheumatoid nodulosis Arthritis Rheum 40:175-178, 1997

Accessory auricle Amnion rupture sequence Syndromes of the Head and Neck, 3rd Edition, pp.11-13, 1990 Branchial cleft cyst – over the sternocleidomastoid muscle (second pharyngeal arch) Cephalocoele – includes encephalocele, meningocoele (rudimentary meningocoele), meningoencephalocele, meningomyelocele; blue nodule with overlying hypertrichosis JAAD 46:934-941, 2002; AD 137:45-50, 2001 Cephalohematoma (cephalohematoma deformans) – blood between outer table of skull and periosteum; fixed Ped Clin North Am 6:1151-1160, 1993 Congenital rhabdomyomatous mesenchymal hamartoma (striated muscle hamartoma) – skin-colored pedunculated (polypoid) nodule of neck, midline of chin, upper lip Ped Derm 16:65-67, 1999; Ped Derm 7:199-204, 1990; Ped Derm 3:153-157, 1986 Craniofacial deformations Syndromes of the Head and Neck, 3rd Edition, pp.1-4, 1990

Dermoid cyst Ectopic thyroid tissue Holoprosencephalic disorders Syndromes of the Head and Neck, 3rd Edition, pp.573-582, 1990 Hydrocephalus Syndromes of the Head and Neck, 3rd Edition, pp.592, 1990 Midline

Encephalocoele Nasal dermoid Nasal glioma

Sialadenitis – in dehydrated neonates Thyroglossal duct cyst and/or sinus – midline cervical mass Ped Clin North Am 6:1151-1160, 1993; J Pediatr Surg 24:966-969, 1989; cleft with sinus tract JAAD 26:885-902, 1992; J Pediatr Surg 19:437-439, 1984 Thyroid gland – goiter; enlarged pyramidal lobe of the thyroid gland Ped Clin North Am 6:1151, 1993

Facial nerve palsy

Paraffinomas – lumpy scalp AD 121:382-385, 1985

Abscesses JAAD 46:934-941, 2002 AIDS – lymphoepithelioid cysts of the parotid glands in AIDS Head Neck 12:337-341, 1990 Actinomycosis Botryomycosis Cervical adenitis Chromomycosis – face, and neck AD 113:1027-1032, 1997; BJD 96:454-458, 1977; AD 104:476-485, 1971 Cryptococcosis – supraclavicular mass Head and Neck 21:239-246, 1999 Lymphadenopathy, reactive – supraclavicular mass Head and Neck 21:239-246, 1999 Mycobacterium tuberculosis – scrofuloderma (cervical scrofula) – infected lymph node, bone, joint, lacrimal gland with overlying red-blue nodule which breaks down, ulcerates, forms fistulae, scarring with adherent fibrous masses which may be fluctuant and draining BJD 134:350-352, 1996; Ped Clin North Am 6:1151-1160, 1993; Head Neck 11:60-66, 1989 Myiasis, furuncular – Dermatobia hominis – scalp cyst in a child Ped Derm 15:116-118, 1998; mimicking ruptured epidermoid cyst Can J Surg 33:145-146, 1990; house fly BJD 76:218-222, 1964; New World screw worm (Cochliomyia), Old World screw worm (Chrysomya), Tumbu fly (Cordylobia) BJD 85:226-231, 1971; black blowflies (Phormia) J Med Entomol 23:578-579, 1986; greenbottle (Lucilia), bluebottle (Calliphora), flesh flies (Sarcophaga, Wohlfartia) Neurosurgery 18:361-362, 1986; rodent botflies (Cuterebra) JAAD 21:763-772, 1989; human botflies (Dermatobia hominis) AD 121:1195-1196, 1985; AD 126:199-202, 1990 Nocardia North American blastomycosis

Onchocercoma Osteomyelitis Periapical abscess Pharyngeal abscess Pott’s puffy tumor – fluctuant nodule over frontal region in patients with chronic sinusitis JAAD 46:934-941, 2002 Pseudomycetoma of scalp – multiple scalp nodules; Trichophyton schoenleinii BJD 145:151-153, 2001 Rhinosporidiosis – vascular nodules of nose, extending to pharynx or lips Mycopathologica 73:79-82, 1981 South American blastomycosis Tinea capitis (T. verrucosum, T. mentagrophtes) – kerion AD 114:371-372, 1978

Langerhans cell histiocytosis, including eosinophilic granuloma

Cervical adenitis Giant cell granuloma Lymphocytoma cutis Cancer 69:717-724, 1992; Acta DV (Stockh) 62:119-124, 1982; Cancer 24:487-502, 1969 Sialadenitis – in neonate due to dehydration

α1 anti-trypsin panniculitis

Cancer of the oral cavity Cheek tag Cylindroma; malignant cylindroma Dermatology 201:255-257, 2000 Cysts of neck

Branchial cleft anomaly (cyst, sinus, and/or fistula) Bronchogenic cyst Dermoid cyst Epidermoid cyst Eruptive vellus hair cysts Heterotopic salivary gland tissue Milia Pilar cyst Steatocystoma multiplex/simplex Thyroglossal duct cyst Teratomas

Dermal dendrocytoma of the face AD 126:689-690, 1990 Epidermoid cyst, including epidermoid cysts of the cranial bones JAAD 46:934-941, 2002; Plast Reconstr Surg 97:1246-1248, 1996; Head Neck 14:415-418, 1992 Eruptive fibromas J Cut Pathol 25 (2):122-125, 1998 Exostosis Extraskeletal Ewing’s sarcoma Ped Derm 5:123-126, 1988 Fibrous dysplasia – side of eyebrow; resembles dermoid

Ganglioneuroma of cervical sympathetic chain Ped Clin North Am 6:1151-1160, 1993 Hibernoma – neck, axilla, central back; vascular dilatation overlying lesion AD 73:149-157, 1956 Infantile myofibromatosis – red scalp nodule AD 123:1391-1396, 1987 Intracranial neoplasms with extension through the skull JAAD 46:934-941, 2002 Kaposi’s sarcoma Otolaryngol Head Neck Surg 111:618-624, 1994; Ann Intern Med 103:744-750, 1985 Keloids Rook p.2056-2057, 1998, Sixth Edition Keratoacanthoma Dermatology 200:317-319, 2000; Otolaryngol Head Neck Surg 93:112-116, 1985; Ann Plast Surg 3:172-176, 1979 Leukemia cutis JAAD 34:375-378, 1996 Lipoma JAAD 46:934-941, 2002 Lymphoma JAAD 46:934-941, 2002; follicular-center B-cell lymphoma – nodules of face, scalp, trunk, extremities BJD 144:1239-1243, 2001; AD 132:1376-1377, 1996; CTCL (fungating ulcerative mass) AD 124:409-413, 1988, Hodgkin’s disease Hodgkin’s disease – ulcerated papules, plaques, and nodules of the scalp and face AD 127:405-410 408, 1991; spindle cell B-cell lymphoma BJD 145:313-317, 2001 Malignant eccrine spiradenoma of the scalp Derm Surg 25:45-48, 1999 Melanocytic nevus – giant cerebriform intradermal nevus Ann Plast Surg 19:84-88, 1987 Merkel cell tumor Metastases to scalp JAAD 46:934-941, 2002; to cervical lymph nodes; supraclavicular masses; breast, uterine, cervical, lung, stomach, oropharyngeal carcinomas Head and Neck 21:239-246, 1999 Mucocoele of lower lip Multiple myeloma Neurofibroma – lateral eyelid; mimics dermoid Neurothekoma – skin-colored scalp nodule BJD 144:1273-1274, 2001 Odontogenic cyst Osteoma Paraganglioma – neck mass Ped Clin North Am 6:1151-1160, 1993 Pilar cyst Plast Reconstr Surg 92:1207-1208, 1993 Pilomatrixoma Pilomatrix carcinoma – multiple of head and neck Otolaryngol Head Neck Surg 109:543-547, 1993; JAAD 23:985-988, 1990 Plasmacytoma Post-auricular cyst Pre-auricular cyst Pre-auricular tag Proliferating trichilemmal tumor of the scalp J Dermatol 27:687-688, 2000; Ann Plast Surg 43:574-575, 1999; Mund Kiefer Gesechtschir 2:216-219, 1998; with malignant transformation Ann Plast Surg 41:314-316, 1998 Rhabdomyosarcoma – neck nodule in children JAAD 31:871-876, 1994; Ped Clin North Am 6:1151-1160, 1993 Salivary gland tumors Sarcoma JAAD 46:934-941, 2002

Schwannoma – supraclavicular mass Head and Neck 21:239-246, 1999 Sebaceoma, giant J Dermatol 21:367-369, 1994 Sebaceous gland carcinoma AD 137:1367-1372, 2001; Nippon Ganka Gakkai Zasshi 104:740-745, 2000 Skin tags Spiradenocarcinoma – vascular scalp nodule Cutis 69:455-458, 2002 Squamous cell carcinoma Derm Surg 28:268-273, 2002; JAAD 23:1174-1175, 1990; JAAD 10:372-378, 1984 Submaxillary cyst Thyroid adenoma, carcinoma, colloid cysts – neck nodules Ped Clin North Am 6:1151-1160, 1993; thyroid carcinoma Head Neck 11:410-413, 1989 Trichoblastoma – scalp Am J Dermatopathol 15:497-502, 1993 Warthin’s tumor, extraparotid – skin-colored neck nodule JAAD 40:468-470, 1999

Adiposis dolorosa Brooke-Spiegler syndrome – trichoepitheliomas and cylindromas of face, scalp, and upper trunk Dermatol Surg 26:877-882, 2000 Cloverleaf skull Syndromes of the Head and Neck, 3rd Edition, pp.536-539, 1990 COH syndrome – cloverleaf skull, polymicrogyria, absent olfactory tracts and bulbs, proptosis, low nasal bridge, short upturned nose, downturned mouth, narrow palate, thumb duplication, small fifth fingers, micropenis, bifid scrotum, agenesis of cervical thymic lobes, bilobed lungs Syndromes of the Head and Neck, 3rd Edition, pp.546-547, 1990 Craniofacial dysostosis Syndromes of the Head and Neck, 3rd Edition, pp.549, 1990 Craniotelencephalic dysplasia Syndromes of the Head and Neck, 3rd Edition, pp.550, 1990 Encephalocutaneous cranial lipomatosis J Paediatr Child Health 36:603-605, 2000; Am J Med Genet 91:261-266, 2000; Ann DV 124:549-551, 1997 Epidermal nevus syndrome Syndromes of the Head and Neck, 3rd Edition, pp.364, 1990 Farber’s disease (disseminated lipogranulomatosis) – red papules and nodules of joints and tendons of hands and feet; deforming arthritis; papules, plaques, and nodules of ears, back of scalp and trunk Rook p.2642, 1998, Sixth Edition; Am J Dis Child 84:449-500, 1952 Fibrodysplasia ossificans progressiva – fibrous scalp nodules Clev Clin Q 51:549-552, 1984 Frontonasal malformation (frontonasal dysplasia, median cleft face syndrome) Syndromes of the Head and Neck, 3rd Edition, pp.785-789, 1990 Gardner’s syndrome – nuchal-type fibroma Am J Surg Pathol 24:1563-1567, 2000; Syndromes of the Head and Neck, 3rd Edition, pp.367, 1990 Great hypertrophy of masseter Infantile myofibromatosis – red to skin-colored nodules AD 134:625-630, 1998 Juvenile hyaline fibromatosis (systemic hyalinosis) – translucent papules or nodules of scalp, face, neck,

trunk, gingival hypertrophy, flexion contractures of large and small joints Ped Derm 18:400-402, 2001; JAAD 16:881-883, 1987 Kawasaki’s disease – cervical adenopathy Ped Clin North Am 6:1151-1160, 1993 Lateral proboscis and cleft lip-palate Syndromes of the Head and Neck, 3rd Edition, pp.775-776, 1990 Lowry syndrome – craniosynostosis with tower skull, ptosis of eyelids, strabismus Syndromes of the Head and Neck, 3rd Edition, pp.555, 1990 Lumpy scalp syndrome – autosomal dominant; irregular scalp nodules, deformed pinnae, rudimentary nipples Clin Exp Dermatol 15:240, 1989 Madelung’s deformity Maffucci’s syndrome Syndromes of the Head and Neck, 3rd Edition, pp.383, 1990 Meckel syndrome – dysencencephalia splanchnocystica Syndromes of the Head and Neck, 3rd Edition, pp.724-727, 1990 Multiple endocrine neoplasia syndrome type II – medullary carcinoma of the thyroid Ped Clin North Am 6:1151-1160, 1993 Neurofibromatosis Syndromes of the Head and Neck, 3rd Edition, pp.395, 1990 Nevoid basal cell carcinoma syndrome (Gorlin’s syndrome) – jaw cysts Syndromes of the Head and Neck, 3rd Edition, pp.373, 1990 Oculo-auriculo-vertebral spectrum (Goldenhaar syndrome) Syndromes of the Head and Neck, 3rd Edition, pp.641-649, 1990 Proteus syndrome – port wine stains, subcutaneous hemangiomas and lymphangiomas, lymphangioma circumscriptum, hemihypertrophy of the face, limbs, trunk; macrodactyly, cerebriform hypertrophy of palmar and/or plantar surfaces, macrocephaly; verrucous epidermal nevi, sebaceous nevi with hyper-or hypopigmentation Am J Med Genet 27:99-117, 1987; vascular nevi, soft subcutaneous masses; lipodystrophy, café au lait macules, linear and whorled macular pigmentation Pediatrics 76:984-989, 1985; Am J Med Genet 27:87-97, 1987; Eur J Pediatr 140:5-12, 1986; Arch Fr Pediatr 47:441-444, 1990 (French) Steatocystoma multiplex

Aneurysmal dilatation of the internal jugular vein – soft blue neck mass Ped Clin North Am 6:1151-1160, 1993 Angiosarcoma (Wilson-Jones angiosarcoma) – nodule or plaque JAAD 40:872-876, 1999; Int J Dermatol 38:697-699, 1999; JAAD 38:143-175, 1998; BJD 136:752-756, 1997; Cancer 59:1046-1057, 1987; multiple nodules BJD 144:380-383, 2001 Arteriovenous malformation JAAD 46:934-941, 2002 Cystic hygroma (lymphatic malformation) Ped Clin North Am 6:1151-1160, 1993; NEJM 309:822-825, 1983 Hemangioma of face and scalp – giant hemangiomas of scalp S Afr Med J 55:47-49, 1979; of face; symptomatic hemangiomas of the airway with airway obstruction and cutaneous hemangiomas in a ‘beard’ distribution J Pediatr 131:643-646, 1997 Lymphangioma

Retiform hemangioendothelioma – exophytic masses of scalp, arms, legs, and penis JAAD 38:143-175, 1998 Thrombus in temporal artery aneurysm Venous malformation – of scalp Zentralbl Neurochir 59:274-277, 1998 Wegener’s granulomatosis

Acquired melanocytic nevus Blue nevus Congenital melanocytic nevus Dysplastic melanocytic nevus Halo nevus Simple lentigo Spitz nevus

Bowenoid papulosis Inverted follicular keratosis Keratoacanthoma Labial lentigo Lichen planus-like keratosis Melanosis of the vulva or penis Pigmented basal cell carcinoma Pigmented actinic keratosis (large cell acanthoma) Pigmented Bowen’s disease Solar lentigo Seborrheic keratosis Squamous cell carcinoma

Angiokeratoma Blue rubber bleb nevus syndrome – mimics metastatic melanoma Hemangioma Kaposi’s sarcoma Pyogenic granuloma Sclerosing hemangioma (dermatofibroma) Thrombosed capillary aneurysm Venous lake

Infarcted acrochordon Chalazion Hemorrhage in the stratum corneum (talon noir)

Nailbed or nailplate hemorrhage Ruptured epidermoid cyst

Acquired localized ochronosis Cosmetic tattoos Foreign body granuloma Mercury injections Minocycline – periungual pigmentation Silver nitrate cautery Silver particles Suture granuloma Tattoos Traumatic (graphite tattoo)

Mycetoma Plantar wart Tinea nigra

Apocrine hidrocystoma Eccrine hidrocystoma Pigmented eccrine poroma Pigmented eccrine porocarcinoma Pigmented pilomatrixoma Plantar pseudochromhidrosis Trichilemmoma

MELANONYCHIA JAAD 21:1165-1175, 1989

NON-NEOPLASTIC

Bacterial subungual infections – Pseudomonas aeruginosa, Klebsiella spp. – Proteus spp. – longitudinal melanonychia Derm Surg 27:580-584, 2001 Bleomycin Blood – longitudinal melanonychia Derm Surg 27:580-584, 2001 Carpal tunnel syndrome Cyclophosphamide Doxorubicin Hydroxyurea JAAD 49:339-341, 2003 5-fluorouracil, topical BJD 112:621-625, 1985 Foreign body (subungual) Friction Dermatologica 174:280-284, 1987

Laugier-Hunziker syndrome – one or more digits JAAD 25:632-636, 1991 Lichen planus BJD 113:369-374, 1985 Longitudinal melanonychia striata – black Nail biting Dermatologica 181:126-128, 1990 Onychomycosis – Trichophyton rubrum JAAD 31:311-316, 1994; Scytalidium dimidiatum Dermatology 202:183-185, 2001 Phaeohyphomycosis of nail – Wangiella dermatitidis Clin Exp Derm 17:83-86, 1992 Post-inflammatory hyperpigmentation Radiotherapy AD 139:1209-1214, 2003 Subungual hemorrhage Ped Derm 21:462-465, 2004; hematoma – longitudinal melanonychia Derm Surg 27:580-584, 2001 Trauma, acute or chronic AD 139:1209-1214, 2003 Verruca vulgaris Zidovudine

NEOPLASTIC

Atypical melanocytic hyperplasia – longitudinal melanonychia Derm Surg 27:580-584, 2001 Basal cell carcinoma JAAD 16:229-233, 1987 Blue nevus – common blue nevus of nail matrix AD 139:1209-1214, 2003 Bowen’s disease Cutis 72:305-309, 2003; JAAD 39:490-493, 1998; JAAD 18:1359-1360, 1988 Fibrous histiocytoma, subungual Keratosis, subungual of the nail bed BJD 140:730-733, 1999 Lentigo simplex – longitudinal melanonychia Derm Surg 27:580-584, 2001 Melanocytic hyperplasia, benign – longitudinal melanonychia Derm Surg 27:580-584, 2001 Melanocytic nevus – longitudinal melanonychia Derm Surg 27:580-584, 2001; nail matrix nevi JAAD 34:765-771, 1996; junctional nevus Ped Derm 21:462-465, 2004; multiple congenital melanocytic nevi JAAD 27:853-854, 1992; spotted grouped pigmented nevi Act DV 56:345-351, 1976; deep penetrating nevus AD 139:1608-1610, 2003; recurrent nevus phenomenon Melanoma Derm Surg 27:294-295, 2001; BJD 143:431-434, 2000; Ann Surg 161:545-552, 1987; Cancer 46:2492-2504, 1980; melanoma in situ AD 140:1102-1106, 2004 Metastatic breast carcinoma Ghatan p.82, 2002, Second Edition Metastatic melanoma Mucous cyst Proliferation of normal melanocytes Postoperative recurrent /persistent melanocytosis

NON-NEOPLASTIC

Acanthosis nigricans Ghatan p.82, 2002, Second Edition AIDS AD 134:1216-1220, 1998; Tyring p.366, 2002; Int J Derm 16:615-630, 1987 Acrotherium nigrum Addison’s disease Ped Derm 21:462-465, 2004

Adrenalectomy for Cushing’s disease Alternaria grisea tenius Amlodipine BJD 153:219-220, 2005 Antimalarials – amodiaquine, quinacrine, chloroquine JAAD 53:S112-114, 2005; Rook p.2831, 1998, Sixth Edition Arsenic poisoning AD 139:1209-1214, 2003 Bacterial subungual infections – Pseudomonas aeruginosa, Klebsiella spp., Proteus spp. – longitudinal melanonychia Derm Surg 27:580-584, 2001 Bacteria coexisting with onychomycosis Blastomycetes Bleomycin JAAD 21:1165-1175, 1989 Busulfan JAAD 21:1165-1175, 1989 Candida Cushing’s syndrome Ghatan p.82, 2002, Second Edition Cyclophosphamide JAAD 21:1165-1175, 1989 Dacarbazine JAAD 53:S112-114, 2005 Daunorubicin Diquat Doxorubicin JAMA 228:460, 1974 Electron beam therapy – transverse melanonychia JAAD 53:S112-114, 2005 5-fluorouracil JAAD 21:1165-1175, 1989 Fluconazole Int J Derm 37:719-720, 1998 Fusarium oxysporum Gold therapy Ped Derm 21:462-465, 2004 Hemochromatosis Ped Derm 21:462-465, 2004 Hemosiderosis Hendersonula toruloidea Hormodendrum elatum Hydroxyurea JAAD 49:339-341, 2003; Int J Derm 39:928-931, 2000 Hyperbilirubinemia Hyperthyroidism Infliximab – transverse melanonychia J Eur Acad DV 16:250, 2002 Irradiation (systemic) Ketoconazole Laugier-Hunziker syndrome J Eur Acad Dermatol Venereol 15:574-577, 2001; Hautarzt 42:512-515, 1991; JAAD 25:632-636, 1991; Clin Exp Derm 15:111-114, 1990 Lichen planus Acta DV 70:338-339, 1990; BJD 113:369-370, 1985 Lichen striatus Lupus erythematosus – diffuse melanonychia JAAD 47:S187-188, 2002 Malnutrition Melphalan Arch Derm Res 258:81-83, 1977 Mepacrine Mercury Methotrexate JAAD 21:1165-1175, 1989 Minocycline Rook p.2852, 1998, Sixth Edition Nail biting and/or picking Dermatologica 181:126-128, 1990 Nitrogen mustard Nitrosourea JAAD 21:1165-1175, 1989

Onychomycosis Rook p.2852, 1998, Sixth Edition Pernicious anemia Ped Derm 21:462-465, 2004 Peutz-Jeghers syndrome Phenolphthalein Ghatan p.82, 2002, Second Edition Phenothiazine Phenytoin Pinta Plaquenil Porphyria Pregnancy AD 139:1209-1214, 2003 Proteus mirabilis Psoralen (PUVA) JAAD 48:S31-32, 2003; Photodermatol 6:98-99, 1989 Racial variation – longitudinal brown streaks of nails; African-American, Hispanic, Indian, Japanese AD 105:548-550, 1972; AD 25:876-881, 1932 Sulfonamide Syphilis, secondary Tetracycline Timolol Trichophyton soudanense Vitamin B12 deficiency X-ray therapy – transverse melanonychia AD 90:174-176, 1964; JAMA 150:210-211, 1952 Zidovudine BJD 126:387-391, 1992

NEOPLASTIC

Breast carcinoma

Allergic contact dermatitis – anonychia Ghatan p.76, 2002, Second Edition Graft vs. host disease – onychoatrophy Textbook of Neonatal Dermatology, p.509, 2001 Epidermolysis bullosa acquisita Rook p.1885, 1998, Sixth Edition Scleroderma Rook p.2529, 1998, Sixth Edition

Congenital hydantoin syndrome Ghatan p.76, 2002, Second Edition

Amyloidosis – primary systemic duVivier; p.529, 2003; J R Soc Med 8:290-291, 1995

Stevens-Johnson syndrome – anonychia (onychoatrophy) Ped Derm 1:298-300, 1984; AD 113:970, 1977

Homocystinuria – arachnodactyly and micronychia Porphyria – congenital erythropoietic porphyria – anonychia Ped Derm 20:498-501, 2003

Epidermolysis bullosa – junctional and dystrophic; anonychia at birth Textbook of Neonatal Dermatology, p.507, 2001; Herlitz type – anonychia Ped Derm 18:217-222, 2001; dominant dystrophic AD 138:269-270, 2002; Rook p.1837, 1998, Sixth Edition Exfoliative dermatitis – anonychia Ghatan p.76, 2002, Second Edition Lichen planus Rook p.1904-1912, 1998, Sixth Edition; AD 96:434-435, 1967

Acrofacial dysostosis Acrogeria – onychoatrophy (anonychia) BJD 142:178-180, 2000; Textbook of Neonatal Dermatology, p.509, 2001 Amniotic bands Textbook of Neonatal Dermatology, p.507, 2001 Anhidrotic ectodermal dysplasia (Clouston syndrome) Cutis 71:224-225, 2003; Textbook of Neonatal Dermatology, p.507, 2001 Ankyloblepharon-ectrodactyly-cleft lip/palate (AEC) syndrome Anonychia congenita Dermatology 200:84-85, 2001; AD 107:752-753, 1973 Anonychia of the thumbnails Textbook of Neonatal Dermatology, p.506, 2001 Anonychia with ectrodactyly Textbook of Neonatal Dermatology, p.506, 2001 Anonychia with flexural pigmentation Textbook of Neonatal Dermatology, p.506, 2001 Anonychia with limb defects Textbook of Neonatal Dermatology, p.506, 2001 Anonychia and microcephaly Am J Med Genet 66:257-260, 1996 Anonychia with onychodystrophy, type B brachydactyly and ectrodactyly Clin Genet 30:219-225, 1986 Anonychia with sensorineural hearing loss (DOOR syndrome – deafness, onycho-osteodystrophy, retardation) Textbook of Neonatal Dermatology, p.506, 2001 Apert syndrome Aplasia cutis congenita Syndromes of the Head and Neck p.417-419 Brachydactyly with nail dysplasia – fourth digit nail absent Brachydactyly type A5 – absence of middle and distal phalanges of hands and feet; fingernail dysplasia 2-5 BJD 152:1339-1342, 2005 Brachydactyly type B – hypoplasia or absence of distal phalanges 2-5 of hands, fingernail hypoplasia of 2-5, with lesser affected toes BJD 152:1339-1342, 2005 Chromosome type C trisomy Chromosome 21 long arm deletion syndrome Coffin-Siris syndrome – anonychia of fifth finger and toenails Am J Dis Child 132:1044, 1978; micronychia Textbook of Neonatal Dermatology, p.507, 2001 Congenital brachydactyly and nail dysplasia – micronychia and anonychia BJD 152:1339-1342, 2005

Congenital onychodysplasia of index fingernails (COIF) (Iso-Kikuchi syndrome) or toenails Ped Derm 16:201-204, 1999; Clin Exp Derm 21:457-458, 1996; J Hand Surg 15A:793-797, 1990; AD 119:8-12, 1983; Ann DV 107:431-435, 1980 Cooks syndrome – hypoplasia/absence of digits 2-5 of hands and feet; hypoplasia of fingernails 1-3, anonychia 4-5; toenail aplasia BJD 152:1339-1342, 2005 Cornelia de Lange syndrome Am J Med Genet 25:163-165, 1986 Cryptophthalmos syndrome (Fraser) Dyskeratosis congenita Textbook of Neonatal Dermatology, p.507, 2001 Ectrodactyly-ectodermal-dysplasia-cleft lip syndrome (EEC syndrome) Clin Genet 3:43-51, 1971 Ellis-van Creveld syndrome (chondroectodermal dysplasia) J Med Genet 17:349-356, 1980 Familial micronychia Fetal alcohol syndrome Textbook of Neonatal Dermatology, p.507, 2001; Drug Alcohol Depend 14:1-10, 1984 Fetal alcohol syndrome Fetal hydantoin syndrome Textbook of Neonatal Dermatology, p.507, 2001; Am J Dis Child 127:758, 1974 Fetal valproate syndrome Teratology 35:465-474, 1987 Fetal warfarin syndrome – nasal hypoplasia, stippled epiphyses Textbook of Neonatal Dermatology, p.507, 2001; J Pediatr 87:838, 1975 Fryns syndrome (cleft palate, diaphragmatic hernia, coarse facies, acral hypoplasia) – anonychia Clin Genet 35:191-201, 1989 Fused digits with micronychia Gingival fibromatosis with ear, nose, bone, nail defects, and hepatosplenomegaly Goltz’s syndrome JAAD 4:273-277, 1981 Hidrotic ectodermal dysplasia Textbook of Neonatal Dermatology, p.507, 2001 Hirschsprung disease with hypoplastic nails and dysmorphic facial features Hutchinson-Gilford syndrome (progeria) – small, thin, dystrophic nails Am J Med Genet 82:242-248, 1999; J Pediatr 80:697-724, 1972 Hypoglossia-hypodactylia syndrome Syndromes of the Head and Neck, p.666-668, 1990 Idiopathic atrophy of the nails Ped Derm 7:39-41, 1990 Keratosis-ichthyosis-deafness (KID) syndrome Laband syndrome (Zimmermann-Laband syndrome) – gingival fibromatosis, aplasia or dysplasia of fingernails, hypertrophy of nasal tip and ears, hypermobility, limb asymmetry Am J Med Genet 31:691-695, 1988; Am J Med Genet 25:543-548, 1986 Nail dysplasia with onychonychia and absence and/or hypoplasia of the distal phalanges Nail-patella syndrome – micronychia Textbook of Neonatal Dermatology, p.507, 2001; Rook p.2833, 1998, Sixth Edition Noonan syndrome Textbook of Neonatal Dermatology, p.507, 2001 Oculodentodigital syndrome Onychotrichodysplasia with neutropenia Oto-onychoperoneal syndrome Eur J Pediatr 138:317-320, 1982 Rapp-Hodgkin ectodermal dysplasia Scleroatrophic and keratotic dermatosis of the limbs Triphalangeal thumbs-onychodystrophy-deafness syndrome Trisomy 3q

Trisomy 8 Textbook of Neonatal Dermatology, p.507, 2001 Trisomy 13 – hypoplastic toenails; micronychia Textbook of Neonatal Dermatology, p.507, 2001 Trisomy 18 – hypoplasia of the 5th finger and toe nails; micronychia Textbook of Neonatal Dermatology, p.507, 2001 Trisomy 21 (Noack’s (Pfeiffer’s) syndrome) – fused digits with micronychia Complete trisomy 22 – primitive low-set ears, bilateral preauricular pit, broad nasal bridge, antimongoloid palpebral fissures, macroglossia, enlarged sublingual glands, cleft palate, micrognathia, clinodactyly of fifth fingers, hypoplastic fingernails, hypoplastic genitalia, short lower limbs, bilateral sandal gap, deep plantar furrows Pediatrics 108:E32, 2001 Turner’s syndrome Textbook of Neonatal Dermatology, p.507, 2001 Williams’ syndrome Witkop tooth-nail syndrome Oral Pathology, Tiecke RW (ed), McGraw-Hill, New York, 1965 Yunis-Varon syndrome – dysplastic clavicles, sparse hair, digital anomalies – anonychia Am J Dis Child 134:649-653, 1980

Polychlorinated biphenyls – natal teeth, pigment anomalies Textbook of Neonatal Dermatology, p.507, 2001

Onychotillomania Physical trauma – anonychia Ghatan p.76, 2002, Second Edition

Digital artery occlusion due to emboli – phalangeal necrosis with onychoatrophy Textbook of Neonatal Dermatology, p.509, 2001 Lymphedema Br J Plast Surg 19:37-42, 1966 Raynaud’s phenomenon – anonychia Ghatan p.76, 2002, Second Edition

MIDLINE FACIAL NODULES OF CHILDREN Abscess Angioma Dermoid cyst Encephalocoele Epidermoid cyst Facial trauma (hematoma, edema) Fibrous dysplasia Hemangioma Histiocytosis Infiltrative tumor (rhabdomyosarcoma) Lymphoma Meningioma Metastatic tumor Nasal glioma Nasolacrimal duct cyst

Neurofibroma Olfactory neuroblastoma Teratoma

MIDLINE NASAL MASSES Ped Derm 17:62-64, 2000; AD 127:362-366, 1991 Abscess Ghatan p.87, 2002, Second Edition Cartilaginous tumor Chondroma Ghatan p.87, 2002, Second Edition Chordoma Ghatan p.87, 2002, Second Edition Dermoid cyst Dermoid sinus Encephalocele (anterior encephalocele) Epidermoid cyst Ethmoidal cyst Fibroma Ghatan p.87, 2002, Second Edition Ganglioneuroma Ghatan p.88, 2002, Second Edition Hemangioma Lacrimal duct cyst Lipoma Meningocoele Metastatic carcinoma Midline malignant B-cell lymphoma Cancer 70:2958-2962, 1992 Nasal glioma Ear Nose Throat J 80:410-411, 2001 Nasal polyp Ghatan p.88, 2002, Second Edition Neurofibroma Ghatan p.87, 2002, Second Edition Olfactory neuroblastoma Ghatan p.87, 2002, Second Edition Papilloma Pilomatrixoma JAAD 51:577-579, 2004; Ghatan p.87, 2002, Second Edition Rhabdomyosarcoma Ghatan p.87, 2002, Second Edition Teratoma of nasal tip

Allergic contact dermatitis Contact Dermatitis 35:49-50, 1996 Bullous pemphigoid Rook p.1869-1870, 1998, Sixth Edition Epidermolysis bullosa acquisita JAAD 44:818-828, 2001; Cutis 32:521-527, 1983 Linear IgA disease Ped Derm 14:303-306, 1997 Lupus erythematosus – bullous LE, resolved JAAD 27:389-394, 1992; Arthritis Rheum 21:58-61, 1978; subacute; discoid LE with milia en plaque BJD 149:424-426, 2003; BJD 137:649-651, 1997

Congenital persistent milia – associated with transverse nasal groove Dermatology 200:151-152, 2000

Epstein’s pearls (milia) in oral cavity of newborns; pearls of areolae, scrotum and labia majora of newborn Rook p.452, 1998, Sixth Edition Primary milia in infants

Acetretin JAAD 37:353-356, 1997; Acta DV 73:235, 1993 Benoxaprofen – pseudoporphyria J R Soc Med 76:525-527, 1983; BJD 106:613, 1982 Corticosteroids, topical JAAD 21:215-217, 1989; AD 122:139-140, 1986 Cyclosporine 5-fluorouracil Ghatan p.257, 2002, Second Edition Penicillamine dermopathy Rook p.3451, 1998, Sixth Edition; AD 125:92-97, 1989

Calcium JAMA 242:455-456, 1979 Liquid nitrogen cryotherapy

Erysipelas, bullous Herpes simplex infection, resolved Herpes zoster scars J Dermatol 23:556-558, 1996 Leprosy Int J Lepr Other Mycobact Dis 62:609-610, 1994 Syphilis – congenital syphilis – palmar and plantar milia JAAD 18:760-771, 1988; Ped Derm 3:395-398, 1986

Amyloidosis, bullous – healing phase AD 124:1683-1686, 1988 Mucinosis – plaque-like erythema with milia in renal transplant patient JAAD 39:334-347, 1998

Calcinosis cutis (pseudomilia) (milia-like calcinosis cutis) Ped Derm 21:483-485, 2004; JAAD 45:152-153, 2001; Eur J Dermatol 10:637-638, 2000; perforating calcinosis cutis J Cutan Pathol 8:247-250, 1981; calcinosis cutis of the penile shaft Genital Skin Disorders, Fischer and Margesson, CV Mosby p.61, 1998 Gout Oxalosis JAAD 31:368-372, 1994 Porphyria – hepatoerythropoietic porphyria AD 138:957-960, 2002; porphyria cutanea tarda – vesicles, bullae, crusts, skin fragility, atrophic scars, milia Rook p.2589-2590, 1998, Sixth Edition; variegate porphyria – as in PCT Rook p.2586-2587, 1998, Sixth Edition; pseudoporphyria Mayo Clin Proc 76:488-492, 2001 Uremia Vitamin D-dependent resistant rickets type IIA – autosomal recessive; hair present at birth then lost in first 12 months; forehead milia; clinically and histologically similar to atrichia with papular lesions AD 141:343-351, 2005

Basaloid follicular hamartoma – yellow plaque with milia AD 129:915-917, 1993 Desmoplastic trichoepithelioma

Eruptive vellus hair cysts Generalized follicular hamartoma AD 131:454-458, 1995 Lymphoma – CTCL JAAD 50:368-374, 2004; pilotropic (follicular) cutaneous T-cell lymphoma BJD 141:315-322, 1999; AD 132:683-687, 1996 Milia, including multiple eruptive milia – face, earlobe Rook p.1669, 1998, Sixth Edition; Ped Derm 16:108-110, 1999; JAAD 37:353-356, 1997; Cutis 60:183-184, 1997; Clin Exp Dermatol 21:58-60, 1996 Nevus depigmentosus with milia BJD 132:317-318, 1995 Pilomatrixomas – multiple eruptive pilomatrixomas Ped Derm 10:382-384, 1993 Syringomas – familial syringomas JAAD 20:702-703, 1989; milia-like syringomas Ped Derm 21:269-271, 2004; JAAD 16:310-314, 1987; eruptive syringomas associated with milia Int J Derm 34:193-195, 1995; perianal milia-like syringomas Dermatology 191:249-251, 1995; syringomas with calcinosis cutis JAAD 23:372-375 Trichoepitheliomas – linear facial plaque with milia – AAD ‘97, Ped Derm section

Chronic actinic damage

Acne-associated milia Alopecia mucinosa (follicular mucinosis) Atrichia with papular lesions – milia of ears and face AD 141:343-351, 2005 Atrophoderma vermiculata Z Hautkr 56:1473-1477, 1981 Darier’s disease Epidermolysis bullosa – dominant dystrophic JID 112:815-817, 1999; generalized atrophic benign epidermolysis bullosa AD 122:704-710, 1986; autosomal recessive epidermolysis bullosa simplex AD 125:931-938, 1989; Dowling-Meara EB Cutis 70:19-21, 2002; AD 122:190-198, 1986; dystrophic epidermolysis bullosa inversa – flexural bullae, oral ulcers, dental caries, milia Ped Derm 20:243-248, 2003; epidermolysis bullosa pruriginosa – reticulate scarring, dermatitis with lichenified plaques, violaceous linear scars, albopapuloid lesions of the trunk, prurigo nodularis-like lesions, milia BJD 152:1332-1334, 2005 Lichen planus Clin Exp Dermatol 24:266-269, 1999; bullous lichen planus Rook p.1904-1912, 1998, Sixth Edition; lichen planus follicularis tumidus with milia and comedones Clin Exp Dermatol 17:346-348, 1992 Lichen sclerosus et atrophicus, bullous BJD 92:711-714, 1975 Milia en plaque Derm Surg 28:291-295, 2002; Int J Derm 39:614-615, 2000; Clin Exp Dermatol 24:183-185, 1999; Cutis 22:67-70, 1978 Primary milia AD 135:1545, 1548, 1999 Transverse nasal line with congenital persistent milia Dermatology 200:151-152, 2000; Clin Exp Derm 18:289-290, 1993 Transient bullous dermolysis of the newborn Ped Derm 20:535-537, 2003; J Cutan Pathol 18:328-332, 1991; JAAD 21:708-713, 1989; AD 121:1429-1438, 1985 Vulvar milia J Dermatol 23:427-428, 1996

Absent dermatoglyphics and transient facial milia JAAD 32:315-318, 1995

Anhidrotic ectodermal dysplasia – milia like lesions with enlarged sebaceous glands on biopsy BJD 149:443-444, 2003 Bart’s syndrome with germ line mosaicism Basaloid follicular hamartoma syndrome – autosomal dominant; multiple skin-colored, red, and hyperpigmented papules of the face, neck chest, back, proximal extremities, and eyelids; syndrome includes milia-like cysts, comedones, dermatosis papulosa nigra, skin tag-like lesions, sparse scalp hair, palmar pits, and parallel bands of papules of the neck (zebra stripes) JAAD 45:644-645, 2001; JAAD 43:189-206, 2000 Bazex-Dupre-Christol syndrome (X-linked dominant) – milia and comedo-like papules, hypotrichosis, follicular atrophoderma, anhidrosis AD 130:337-342, 1994; Ped Derm 16:108-110, 1999; Ann Dermatol Syphilgr (Paris) 93:241-254, 1966 Bazex-Dupre-Christol-like syndrome – basal cell carcinomas, hypohidrosis, hypotrichosis, milia Derm Surg 26:152-154, 2000; AD 130:337-342, 1994 Congenital hypotrichosis and milia Am J Med Genet 56:423-424, 1995 Down’s syndrome – idiopathic milia-like calcinosis cutis Ped Derm 19:271-273, 2002; JAAD 45:152-153, 2001; BJD 134:143-146, 1996; JAAD 32:129-130, 1995; AD 125:1586-1587, 1989; perforating milia-like calcinosis with syringomas in Down’s syndrome Ped Derm 11:258-260, 1994 Extensive reticular hyperpigmentation and milia Ped Derm 16:108-110, 1999 Familial multiple eruptive milia Birth Defects 7:333-337, 1971; Ped Derm 16:108-110, 1999 Hereditary perioral pigmented follicular atrophoderma with milia-like epidermoid cysts BJD 139:713-718, 1998 Hypotrichosis with light-colored hair and facial milia Ped Derm 16:108-110, 1999 Marie-Unna type congenital hypotrichosis Rook p.452, 1998, Sixth Edition Milia with nodular calcinosis and palmoplantar keratoderma Ann Dermatol Venereol (Paris) 107:273-277, 1980 Multiple follicular hamartomas with sweat gland and sebaceous differentiation, vermiculate atrophoderma, milia, hypotrichosis, and late development of basal cell carcinomas JAAD 39:853-857, 1998 Multiple trichoepitheliomas, cylindromas, and milia Ann DV 114:175-182, 1987 Nevoid basal cell carcinoma syndrome BJD 145:508-509, 2001; JAAD 42:939-969, 2000; with palmar epidermoid cyst, milia, and maxillary cysts BJD 145:508-509, 2001 Nicolau and Balus syndrome – syringomas, milia, and atrophoderma vermiculata Dermatologica 162:281-286, 1981 Oral-facial-digital syndrome type 1 Am J Med Genet 86:269-273, 1999; JAAD 31:157-190, 1994; Ped Derm 9:52-56, 1992 Pachyonychia congenita, Jackson-Lawler type (type 2) – white milia-like cysts at birth JAAD 38:1007-1009, 1998 Persistent milia, steatocystoma multiplex and eruptive vellus hair cysts within a family Dermatology 196:392-396, 1998 Pseudoxanthoma elasticum with milia en plaque J Cutan Pathol 24:61-63, 1997 Rasmussen syndrome – trichoepitheliomas, milia, and cylindromas AD 111:610, 1975; with palmoplantar keratoderma Ped Derm 16:108-110, 1999 Reticular pigmented genodermatosis with milia (Naegeli-Franceschetti-Jadassohn syndrome?) Clin Exp Dermatol 20:331-335, 1995

Rombo syndrome – papules and cysts of the face and trunk, basal cell carcinomas, vermiculate atrophoderma, milia, hypotrichosis, trichoepitheliomas, peripheral vasodilatation with cyanosis BJD 144:1215-1218, 2001; JAAD 39:853-857, 1998; Acta DV 61:497-503, 1981 Trichoepitheliomas and milia Ped Derm 16:108-110, 1999

Autologous skin transplants Br J Plast Surg 6:153, 1950 Burns, second degree Carbon dioxide laser resurfacing Dermabrasion J Derm Surg Oncol 14:1301, 1988; AD Syphilol 68:589, 1953 Physical trauma Post-radiation therapy Surgical scar X-rays

Congenital hemangioma with milia-like structures Ped Derm 15:307-308, 1998

GENERALIZED

Hereditary progressive mucinous histiocytosis AD 130:1300, 1994 Lichen myxedematosus (scleromyxedema) Mucopolysaccharidoses – Hunter’s, Hurler’s, Schei, Sly syndromes Myxedema (generalized, pretibial) Scleredema

LOCALIZED

Acral persistent papular mucinosis JAAD 21:293, 1989 Angiomyxoma Ped Derm 20:230, 2003 Congenital linear cutaneous mucinosis of infancy AD 122:790, 1986 Cutaneous focal mucinosis (digital mucous or myxoid cyst, extradigital cutaneous myxoma) Cutaneous mucinosis of infancy JAAD 24:265-270, 1991 Cutaneous myxomas, spotty pigmentation, endocrine overactivity AD 122:790, 1986 Discrete papular lichen myxedematosus Cutis 75:105-112, 2005 Juvenile hyaline fibromatosis Follicular mucinosis (alopecia mucinosa) Lipoid proteinosis Mucinous nevus Ped Derm 20:230, 2003 Neuropathia mucinosa cutanea Ped Derm 20:230, 2003 Nodular lichen myxedematosus Cutis 75:105-112, 2005 Pachydermoperiostosis Plaque-like cutaneous mucinosis AD 130:1433-1438, 1994 Pretibial myxedema

Reticular erythematous mucinosis Self-healing juvenile cutaneous mucinosis

Degos’ disease Dermatomyositis Eosinophilia-myalgia syndrome associated with L-tryptophan ingestion Fibrocytic-histiocytic proliferations (dermatofibroma, fibromatosis, nodular fasciitis, atypical fibroxanthoma) HIV disease and papular mucinosis AD 128:996-997, 1992 Hypothyroidism – focal mucinosis Cutis 32:449, 1983 Toxic oil syndrome – ingestion JAAD 16:139-140, 1987 Lupus erythematosus – papulonodular mucinosis associated with systemic lupus erythematosus; plaque-like cutaneous lupus mucinosis AD 129:383, 1993 Mucinosis of obesity (localized lichen myxedematosus (papular mucinosis) in morbid obesity) BJD 148:165-168, 2003 Neoplasms (neurofibroma, neurilemmoma, adenoid basal cell carcinoma, adnexal carcinomas) Nephrogenic fibrosing dermopathy Am J Med 114:563-572, 2003; scleromyxedema-like cutaneous disease in renal dialysis patients) Lancet 356:1000-1001, 2000 Ultraviolet light and PUVA therapy

Allergic rhinitis Pemphigus vegetans Ghatan p.88, 2002, Second Edition Pemphigus vulgaris Cutis 34:394-395, 1984 Rheumatoid nodule – nasal nodule JAAD 53:191-209, 2005; JAAD 8:439-457, 1983

Dermoid cyst AD 135:463-468, 1999 Dermoid sinus Dermatol Therapy 18:104-116, 2005 Embryonal rhabdomyosarcoma – orbit, nasopharynx, nose Rook p.2369, 1998, Sixth Edition Encephalocele Textbook of Neonatal Dermatology, p.121, 2001 Epidermoid cyst – congenital midline mass Soc Ped Derm Annual Meeting, Poster Session, July 2005 Hemangioma congenital midline mass Soc Ped Derm Annual Meeting, Poster Session, July 2005 Lacrimal duct cyst Lymphoma – congenital midline mass Soc Ped Derm Annual Meeting, Poster Session, July 2005 Meningocoele Meningoencephalocoele Meningioma – extracranial meningioma Metastatic tumor – congenital midline mass Soc Ped Derm Annual Meeting, Poster Session, July 2005

Nasal glioma – red or bluish nodules; resemble hemangiomas, nasal encephalocoele, meningoencephalocoele, extracranial meningioma, dermoid cyst, lacrimal duct cyst, neuroblastoma, rhabdomyosarcoma Ear Nose Throat J 80:410-411, 2001; Rook p.603, 1998, Sixth Edition Nasal midline masses Dermatol Therapy 18:104-116, 2005; AD 127:362-366, 1991 Neuroblastoma – olfactory neuroblastoma Soc Ped Derm Annual Meeting, Poster Session, July 2005 Neurofibroma – congenital midline mass Soc Ped Derm Annual Meeting, Poster Session, July 2005 Rhabdomyosarcoma – congenital midline mass Soc Ped Derm Annual Meeting, Poster Session, July 2005 Subcutaneous abscess – congenital midline mass Soc Ped Derm Annual Meeting, Poster Session, July 2005 Teratoma of nasal tip Ann Plast Surg 35:522-524, 1995 Trauma, facial congenital midline mass Soc Ped Derm Annual Meeting, Poster Session, July 2005

Silicone granulomas – nasal enlargement JAAD 52:S53-56, 2005 Smoking-induced nasopharyngeal lymphoid hyperplasia Laryngoscope 107:1635-1642, 1997

Abscess Actinomycosis – indurated red nose JAAD 38:310-313, 1998 Aspergillosis Oral Surg Oral Med Oral Pathol 59:499-504, 1985 Candidiasis – chronic mucocutaneous candidiasis Chromomycosis Cryptococcosis – indurated red nose JAAD 38:310-313, 1998 Erysipelas Fusarium – of sinuses; nasal erythema with conjunctivitis JAAD 47:659-666, 2002 Granuloma inguinale Herpes simplex virus Herpes zoster Histoplasmosis – indurated red nose JAAD 38:310-313, 1998; Mycopathologia 115:13-18, 1991 Leishmaniasis – mucocutaneous leishmaniasis; indurated red nose JAAD 38:310-313, 1998; Am J Trop Med Hyg 59:49-52, 1998; Leishmania aethiopica – nasal infiltration with edema but no destruction Trans R Soc Trop Med Hyg 63:708-737, 1969; mucocutaneous leishmaniasis with destruction of the nose and midface JAAD 34:257, 1996 Leprosy – indurated red nose JAAD 38:310-313, 1998; Rook p.1224, 1998, Sixth Edition Mycobacterium avium-intracellulare – nasal papules JAAD 33:528-531, 1995 Mycobacterium tuberculosis – lupus vulgaris; indurated red nose JAAD 38:310-313, 1998; starts as red-brown plaque; vegetating forms – ulcerate, areas of necrosis, invasion of mucous membranes with destruction of cartilage (lupus vorax); tumor-like forms – deeply infiltrative; soft smooth nodules or red-yellow hypertrophic plaque; nasal papules; Rook p.1196, 1998, Sixth Edition; Int J Dermatol 26:578-581, 1987; Acta Tuberc Scand 39 (Suppl 49):1-137, 1960 Nasal septum abscess

North American blastomycosis – indurated red nose JAAD 38:310-313, 1998; Laryngoscope 103:53-58, 1993 Paracoccidiodomycosis – indurated red nose JAAD 38:310-313, 1998 Pinta Rhinoscleroma – Klebsiella rhinoscleromatis (Frisch bacillus) exudative stage with rhinorrhea; then proliferative stage with exuberant friable granulation tissue of nose (Hebra nose), pharynx, larynx; progresses to nodules; then fibrotic stage Acta Otolaryngol 105:494-499, 1988; Cutis 40:101-103, 1987; indurated red nose JAAD 38:310-313, 1998 Rhinosporidiosis – soft polyps with minute white spots; Indian sand dredgers Ghatan p.144, 2002, Second Edition Sinusitis, acute Sporotrichosis – indurated red nose JAAD 38:310-313, 1998; fixed cutaneous Staphylococcus aureus – nasal carriage with intranasal folliculitis or vestibulitis Subcutaneous zygomycosis (Conidiobolus) – very disfiguring JAAD 30:904-908, 1994 Syphilis Verruga peruana Yaws – proliferative osseous form (goundou) Rook p.1149,1270, 1998, Sixth Edition Zygomycosis (Basidiobolus coronatus) AD 141:1211-1213, 2005

Juvenile xanthogranuloma Langerhans cell histiocytosis Lichen myxedematosus

Crohn’s disease J Otolaryngol 14:399-400, 1985 Lymphocytoma cutis – idiopathic or due to Lyme borreliosis JAAD 38:877-905, 1998 Lymphoid hyperplasia of the nasal cavity in AIDS Ann Otolaryngol Chir Cervicofac 105:543-547, 1988 Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) J Cutan Pathol 25:563-567, 1998; BJD 134:749-753, 1996 Sarcoid – rhinophymatous sarcoid J Drugs Dermatol 3:333-334, 2003; Rook p.1149,2108,2694, 1998, Sixth Edition; lupus pernio JAAD 39:835-838, 1998; Lupus 1:129-131, 1992; JAAD 22:439-443, 1990

Acromegaly

Aggressive intranasal carcinoma – edema and erythema of nose Cutis 42:288-293, 1988 Angiosarcoma – indurated red nose JAAD 38:310-313, 1998; JAAD 38:837-840, 1998; resembling rhinophyma JAAD 49:530-531, 2003 Basal cell carcinoma mimicking rhinophyma – indurated red nose JAAD 38:310-313, 1998

Carcinoma of the nasal columella Am J Surg 170:453-456, 1995 Carcinoma of the nasal vestibule Int J Radiat Oncol Biol Phys 10:627-637, 1984 Cellular neurothekoma – papule Ped Derm 12:191-194, 1995 Epidermoid cyst Folliculosebaceous cystic hamartoma – nasal nodule JAAD 34:77-81, 1996 Kaposi’s sarcoma – enlargement of tip of nose in AIDS Rook p.1063,1998, Sixth Edition; mimicking pyogenic granuloma of the nasal mucosa J Laryngol Otol 112:280-282, 1998 Keratoacanthoma Lacrimal duct cyst Leukemia J Laryngol Otol 106:261-263, 1992 Lymphoma Rook p.1149, 1998, Sixth Edition; JAAD 38:310-313, 1998; Br J Haematol 97:821-829, 1997; primary cutaneous B-cell lymphoma – rhinophyma-like enlargement of nose AD 140:751-756, 2004; midline malignant B-cell lymphoma Cancer 70:2958-2962, 1992; angiocentric T-cell lymphoma JAAD 26:31-38, 1992; nasal type CD56+ natural killer cell/T-cell lymphoma BJD 142:1021-1025, 2000; primary cutaneous follicular center cell lymphoma JAAD 52:S73-75, 2005 Malignant eccrine spiradenoma Dermatol Surg 27:417-420, 2001 Malignant histiocytosis J Clin Pathol 35:599-605, 1982 Melanocytic nevus, congenital Meningioma Merkel cell carcinoma – reddish-blue nodules; legs, lip, eyelid, scalp, nose Histopathology 7:229-249, 1983 Metastasis – rhinophyma-like metastatic carcinoma Cutis 57:33-36, 1996 Nasal septal carcinoma – mimicking rosacea J Derm Surg 13:1021-1024, 1987 Nasopharyngeal carcinoma Laryngoscope 111:645-649, 2001 Neuroblastoma Minim Invasive Neurosurg 44:79-84, 2001 Neurofibroma Osteosarcoma of the orbit Ophthal Plast Reconstr Surg 14:62-66, 1998 Papilloma Plasmacytosis, mucocutaneous Polyps Allergy 54 Suppl 53:7-11, 1999 Rhabdomyosarcoma Schwannoma No Shinkei Geka 20:1189-1194, 1992. Japanese Sebaceous adenoma Sebaceous carcinoma Sebaceous hyperplasia Striated muscle hamartoma of the nostril J Dermatol 22:504-507, 1995 Squamous cell carcinoma Trichoepitheliomas, multiple BJD 95:225-232, 1976

Acrokeratosis paraneoplastica (Bazex syndrome) Cutis 55:233-236, 1995; Medicine 70:269-280, 1991

Acne necrotica varioliformis Alopecia mucinosa

Darier’s disease Granuloma faciale BJD 153:851-853, 2005; Am J Otolaryngol 4:184-186, 1983 Granulosis rubra nasi G Ital DV 125:275-276, 1990 Malignant pyoderma AD 122:295-302, 1986 Rhinophyma – acne rosacea Rook p.2104-2110,3581, 1998, Sixth Edition

Bonnet-Dechaume-Blanc syndrome – midfacial arteriovenous malformation Textbook of Neonatal Dermatology, p.329, 2001 Brancho-oculo-facial syndrome – bulbous nose Ped Derm 12:24-27, 1995 Costello’s syndrome – nasal papillomas Ped Derm 11:277-279, 1994 Fibropapule multiplex of the nose – a possible variant of Cowden’s disease Dermatology 192:379-381, 1996 Fibrous dysplasia Hereditary progressive mucinous histiocytosis JAAD 35:298-303, 1996 Juvenile hyaline fibromatosis – bulbous nose Ped Derm 6:68-75, 1989; Am J Med Genet 26:123-131, 1987 Laband syndrome J Oral Pathol Med 19:385-387, 1990 Lipoid proteinosis – papules Ped Derm 9:264-267, 1992 Mucopolysaccharidoses Muir-Torre syndrome Multicentric reticulohistiocytosis Hautarzt 46:118-120, 1995 Proteus syndrome J Craniofac Surg 6:151-160, 1995 Pseudohypoparathyroidism – depressed nasal bridge Ped Derm 9:11-18, 1993 Steatocystoma multiplex, congenital linear Ped Derm 17:136-138, 2000 Trichorhinophalangeal syndrome – bulbous nose Ped Derm 10:385-387, 1993

Trauma – nasal fracture

Angioendotheliosarcoma Acta DV 64:88-90, 1984 Angiofibromas (tuberous sclerosis) Angiomatous nevus Arteriovenous malformation Bolognia p.1625, 2003 Hemangioma – diffuse hemangioma AD 139:869-875, 2003; Rook p.556, 1998, Sixth Edition Wegener’s granulomatosis Rook p.1149, 1998, Sixth Edition

Ped Derm 17:62-64, 2000; AD 127:362-366, 1991 Abscess Ghatan p.87, 2002, Second Edition Cartilaginous tumor Chondroma Ghatan p.87, 2002, Second Edition Chordoma Ghatan p.87, 2002, Second Edition Dermoid cyst Dermoid sinus

Encephalocele (anterior encephalocele) Epidermoid cyst Ethmoidal cyst Fibroma Ghatan p.87, 2002, Second Edition Ganglioneuroma Ghatan p.88, 2002, Second Edition Hemangioma Lacrimal duct cyst Lipoma Meningocoele Metastatic carcinoma Midline malignant B-cell lymphoma Cancer 70:2958-2962, 1992 Nasal glioma Ear Nose Throat J 80:410-411, 2001 Nasal polyp Ghatan p.88, 2002, Second Edition Neurofibroma Ghatan p.87, 2002, Second Edition Olfactory neuroblastoma Ghatan p.87, 2002, Second Edition Papilloma Pilomatrixoma JAAD 51:577-579, 2004; Ghatan p.87, 2002, Second Edition Rhabdomyosarcoma Ghatan p.87, 2002, Second Edition Teratoma of nasal tip

Lupus erythematosus, systemic Arch Otolaryngol 99:456-457, 1974 Mixed connective tissue disease

Nasal aplasia, heminasal aplasia with or without proboscis Syndromes of the Head and Neck, p.585-586, 1990 Noma neonatorum – deep ulcers with bone loss, mutilation of nose, lips, intraorally, anus, genitalia; Pseudomonas, malnutrition, immunodeficiency Textbook of Neonatal Dermatology, p.149, 2001

Trigeminal trophic syndrome (Wallenberg’s syndrome) JAAD 50:549-552, 2004

Acoustic neuroma Alcohol or glycerol injection of gasserian ganglion Astrocytoma Cortical and brainstem infarcions Herpes zoster ophthalmicus Intracranial meningioma Mycobacterium leprae neuritis Postencephalitic parkinsonism Posterior, inferior cerebellar artery insufficiency Spinal cord degeneration Syringobulbia Trauma Trigeminal rhizotomy Vertebrobasilar insufficiency

Corticosteroids, topical J Pediatr 105:840-841, 1984 Sweet’s syndrome, drug-induced – red plaques, nasal ulcers, perianal ulcers – celecoxib JAAD 45:300-302, 2001

Button batteries – foreign body in the nose J Otolaryngol 21:458-460, 1992 Chrome plating Occup Med (Lond) 48:135-137, 1998 Cocaine abuse – cocaine sniffing ulcer NEJM 352:609-615, 2005; Cutis 65:73-76, 2000; mimicking midline granuloma JAAD 32:286-287, 1995

Alternariosis Anthrax JAAD 50:549-552, 2004 Aspergillosis JAAD 50:549-552, 2004 Balamuthia mandrillaris (granulomatous amebic infection) – central facial destruction JAAD 50:S38-41, 2004 Cancrum oris (noma) – nasopharyngeal mutilation J Craniofac Surg 12:273-283, 2001 Coccidioidomycosis Cryptococcosis Cytomegalovirus Glanders – Pseudomonas mallei – cellulitis which ulcerates with purulent foul-smelling discharge, regional lymphatics become abscesses; nasal and palatal necrosis and destruction and mutilation; metastatic papules, pustules, bullae over joints and face, then ulcerate; deep abscesses with sinus tracts occur; polyarthritis, meningitis, pneumonia Rook p.1146-1147, 1998, Sixth Edition Herpes simplex virus Histoplasmosis NEJM 352:609-615, 2005; Mycopathologia 115:13-18, 1991 Leishmaniasis – espundia (mucocutaneous leishmaniasis) – nasopharyngeal ulceration and/or mutilation NEJM 352:609-615, 2005; Am J Trop Med Hyg 59:49-52, 1998 Leprosy – lepromatous leprosy – misshapen nose with collapse of nose and saddle nose deformity NEJM 352:609-615, 2005; Rook p.1224, 1998, Sixth Edition; primary diffuse lepromatous leprosy (la lepra bonita) JAAD 51:416-426, 2004; leprous trigeminal neuritis – nasopharyngeal mutilation Rook p.1149, 1998, Sixth Edition Mucormycosis JAAD 50:549-552, 2004 Mycobacterium africanum Clin Inf Dis 21:653-655, 1995 Mycobacterium tuberculosis – lupus vulgaris; vegetating forms – ulcerate, areas of necrosis, invasion of mucous membranes with destruction of cartilage (lupus vorax); saddle nose deformity; nasal involvement with friable nodules which ulcerate NEJM 352:609-615, 2005; Rook p.1149, 1998, Sixth Edition; Int J Dermatol 26:578-581, 1987; Acta Tuberc Scand 39 (Suppl 49):1-137, 1960 Myiasis J Dermatol 22:348-350, 1995 North American blastomycosis – nasopharyngeal mutilation Laryngoscope 103:53-58, 1993 Paracoccidioidomycosis – nasopharyngeal ulceration and/or mutilation NEJM 352:609-615, 2005; JAAD 50:549-552, 2004 Rhinoentomophthoromycosis JAAD 50:S38-41, 2004 Rhinoscleroma – nasopharyngeal mutilation NEJM 352:609-615, 2005; Ped Derm 21:134-138, 2004; Acta

Otolaryngol 105:494-499, 1988; Cutis 40:101-103, 1987; Wien Med Wochenschr 20:1-5, 1870 Rhinosporidiosis – nasopharyngeal mutilation NEJM 352:609-615, 2005 Sporotrichosis JAAD 50:549-552, 2004 Syphilis – congenital – destruction of nasal septum Rook p.1252, 1998, Sixth Edition; tertiary (gumma) NEJM 352:609-615, 2005; Tyring p.329, 2002; Rook p.1149, 1998, Sixth Edition; endemic (bejel) – nasopharyngeal mutilation Yaws – nasopharyngeal mutilation (gangosa) Rook p.1149,1270-1271, 1998, Sixth Edition

Crohn’s disease Ear Nose Throat J 79:520-523, 2000 Lethal midline granuloma – nasopharyngeal mutilation Rook p.1149, 1998, Sixth Edition Pyoderma gangrenosum BJD 141:1133-1135, 1999 Sarcoid, ulcerative – nasopharyngeal mutilation NEJM 352:609-615, 2005; Dermatology 199:265-267, 1999; JAAD 39:835-838, 1998; BJD 99 (Suppl.16):54-55, 1978

Porphyria – congenital erythropoietic porphyria – mutilation of the nose due to photosensitivity Semin Liver Dis 2:154-63, 1982 Prolidase deficiency – autosomal recessive; saddle nose deformity; skin spongy and fragile with annular pitting and scarring; leg ulcers; photosensitivity, telangiectasia, purpura, premature graying, lymphedema Ped Derm 13:58-60, 1996; JAAD 29:819-821, 1993; AD 127:124-125, 1991; AD 123:493-499, 1987

Adenocarcinoma Basal cell carcinoma NEJM 352:609-615, 2005; Rook p.1681-1683, 1998, Sixth Edition; Acta Pathol Mibrobiol Scand 88A:5-9, 1980 Chondrosarcoma Lymphoma, including nasal NK T-cell lymphoma (nasal lymphoma, polymorphic reticulosis, lymphomatoid granulomatosis, and malignant midline granuloma) JAAD 52:708-709, 2005; AD 133:1156-1157, 1997; angiocentric lymphoma Cancer 66:2407-2413, 1990 Malignant epithelial and mesenchymal tumors (sarcomas) – nasopharyngeal mutilation Malignant histiocytosis – mimicking lethal midline granuloma Pathol Res Pract 171:314-324, 1981 Melanoma Nasal polyps – nasopharyngeal mutilation Squamous cell carcinoma NEJM 352:609-615, 2005; associated with HPV-5 in epidermodysplasia verruciformis Tyring p.278, 2002

Eosinophilic angiocentric fibrosis (variant of granuloma faciale) – red facial plaque with saddle nose deformity BJD 152:574-576, 2005; Histopathology 9:1217-1225, 1985 Hydroa vacciniforme – saddle nose deformity Ped Derm 21:555-557, 2004

Factitial dermatitis JAAD 50:549-552, 2004

Anhidrotic ectodermal dysplasia – saddle-nose deformity Ghatan p.264, 2002, Second Edition Anti-phospholipid antibody syndrome – nasal tip necrosis BJD 142:1199-1203, 2000 Hurler’s syndrome – saddle-nose deformity Ghatan p.264, 2002, Second Edition Hyper-IgE syndrome J Invest Allergol Clin Immunol 3:217-220, 1993 Relapsing polychondritis – saddle nose deformity, red ears, and airway collapse NEJM 352:609-615, 2005; Rook p.2042, 1998, Sixth Edition; Chest 91:268-270, 1987; Medicine 55:193-216, 1976

Alkaline dusts (soap powders) Anhydrous sodium carbonate (soda ash) Arsenic Bromoderma – nasopharyngeal mutilation Capsaicin (active agent in capsicum) Chromium inhalation – chrome ulcers Am J Ind Med 26:221-228, 1994 Copper salts Dimethyl sulfate Fluorides Iododerma – nasopharyngeal mutilation Lime Mercury organic compounds Snuff

Digital trauma Recurrent cautery Surgery – trophic ulcer following surgery – nasopharyngeal mutilation

Churg-Strauss syndrome Otolaryngol Head Neck Surg 88:85-89, 1980 Hemangioma, infantile Rook p.556, 1998, Sixth Edition Vasculitis Vascular neoplasm Wegener’s granulomatosis NEJM 352:609-615, 2005; NEJM 352:392, 2005; Rook p.1149,2218, 1998, Sixth Edition; AD 130:861-867, 1993

Allergic contact dermatitis – cosmetics, nail polish Contact Dermatitis 34:140-141, 1996; fragrances, necklaces Rook p.786, 1998, Sixth Edition; Compositae dermatitis – lichenified dermatitis along posterior hairline Rook p.788, 1998, Sixth Edition; airborne allergic contact dermatitis JAAD 15:1-10, 1986

Atopic dermatitis – poikiloderma-like lesions of the neck J Dermatol 17:85-91, 1990 Autoimmune progesterone dermatitis Bullous pemphigoid Dermatitis herpetiformis Dermatomyositis – erythema of neck Rook p.2559-2560, 1998, Sixth Edition Epidermolysis bullosa acquisita Graft vs. host disease, acute – papules Taiwan I Hsueh Hui Tsa Chih 88:657-672, 1989 Hyper-IgE syndrome – papular, pustular, excoriated dermatitis of scalp, buttocks, neck, axillae, groin; furunculosis; growth failure Clin Exp Dermatol 11:403-408, 1986; Medicine 62:195-208, 1983 Linear IgA disease Lupus erythematosus – systemic LE; tumid lupus JAAD 41:250-253, 1999; Am J Dermatopathol 21:356-360, 1999; bullous dermatosis of SLE (annular bullae) – face, neck, upper trunk, oral bullae JAAD 27:389-394, 1992; Ann Intern Med 97:165-170, 1982; Arthritis Rheum 21:58-61, 1978; discoid lupus, subacute cutaneous lupus erythematosus – annular and polycyclic lesions Med Clin North Am 73:1073-1090, 1989; JAAD 19:1957-1062, 1988 Morphea Pemphigus foliaceus of children – arcuate, circinate, polycyclic lesions JAAD 46:419-422, 2002; Ped Derm 3:459-463, 1986 Rheumatoid neutrophilic dermatitis AD 133:757-760, 1997 Scleroderma Still’s disease Rook p.2570, 1998, Sixth Edition Urticaria

Dermatol Therapy 18:104-116, 2005 Branchial cleft cyst, sinus and/or fistula; overlying anterior border of the sternocleidomastoid muscle Int J Oral Maxillofac Surg 25:449-452, 1996; Int J Dermatol 19:479-486, 1980; confused with malignant or tuberculous lymphadenopathy, parotid or thyroid tumor, thymopharyngeal cyst, thyroglossal cyst, dermoid cyst, teratoma, carotid body tumor, hemangioma, neurofibroma Plastic Reconstruct Surg 100:32-39, 1997; Arch Otolaryngol Head Neck Surg 123:438-441, 1997; J Pediatr Surg 24:966-969, 1989; Melnick-Fraser syndrome – preauricular pits, hearing loss, and renal anomalies Branchial cleft sinus – pit in lower third of the neck along anterior border of sternocleidomastoid muscle; skin tag at opening Clin Otolaryngol 3:77-92, 1978 Branchial vestige (wattles) (cervical chondrocutaneous branchial remnants) – neck nodule in children – remnants include cysts (deep to middle or lower thirds of sternocleidomastoid muscles), sinus tracts, and cartilagenous remnants (overlying anterior border of sternocleidomastoid muscles); pinpoint firm masses or ear-like projections; (accessory tragus) (wattle or cervical auricle) AD 134:499-504, 1998; Ped Clin North Am 6:1151-1160, 1993; JDSO 7:39-41, 1981 Bronchogenic cyst – keratotic papule, sinus tract or cyst at the suprasternal notch; neck, shoulders, back and chest AD 136:925-930, 2000; Ped Derm 15:277-281, 1998; Ped Derm 12:304-306, 1995; J Cutan Pathol 12:404-409, 1985 Cartilaginous rest of the neck (wattle) Cutis 58:293-294, 1996; Int J Derm 25:186-187, 1986; AD 127:404-409, 1985 Cervical braid Br J Plastic Surg 43:369-370, 1990 Cervical tab Cervicothymic cyst – resembles branchial cleft cyst

Congenital rhabdomyomatous mesenchymal hamartoma (striated muscle hamartoma) – skin-colored pedunculated (polypoid) nodule of neck, midline of chin, upper lip Ped Derm 16:65-67, 1999; Ped Derm 7:199-204, 1990; Ped Derm 3:153-157, 1986 Cystic hygromas Cystic teratoma – large neck mass Dermoid cyst – neck,midline Ped Clin North Am 6:1151, 1993; midline of nose, lateral eyebrow, scrotum, sternum, perineal raphe, and sacral areas Curr Prob Derm 8:137-188, 1996; Acta Neurochir (Wien) 128:115-121, 1994 Ectopic respiratory epithelium – red plaque of the neck BJD 136:933-934, 1997 Ectopic thyroid tissue Ped Clin North Am 6:1151-1160, 1993 Esophageal diverticulum Ghatan p.241, 2002, Second Edition Fourth branchial sinus causing recurrent cervical abscess Aust N Z J Surg 67:119-122, 1997 Laryngocele Ghatan p.241, 2002, Second Edition Midline cervical clefts – vertically oriented atrophic area of lower anterior neck, associated skin tags or sinus tracts, fibrous bands connect to platysma muscle Ped Derm 17:118-122, 2000; Int J Derm 19:479-486, 1980 Nevus simplex (capillary ectasias) – glabella, eyelids, nose, upper lip, nape of neck Eichenfeld p.100, 2001 Occult spinal dysraphism – papule Ped Derm 12:256-259, 1995 Pterygium colli medianum Rhabdomyomatous mesenchymal hamartoma – pedunculated papule associated with a midline cervical cleft AD 141:1161-1166, 2005 Teratoma – bulky tumors of newborn Ped Clin North Am 6:1151-1160, 1993 Thymopharyngeal cyst Thyroglossal duct cyst and/or sinus – midline cervical mass Ped Clin North Am 6:1151-1160, 1993; J Pediatr Surg 24:966-969, 1989; cleft with sinus tract JAAD 26:885-902, 1992; J Pediatr Surg 19:437-439, 1984 Thyroid gland – enlarged pyramidal lobe of the thyroid gland Ped Clin North Am 6:1151, 1993

Cyclosporine – acne keloidalis nuchae BJD 143:465-466, 2000 Penicillamine – elastosis perforans serpiginosa AD 138:169-171, 2002 Protease inhibitor (saquinavir, nelfinavir, indinavir) – buffalo hump JAAD 46:284-293, 2002

Chrysiasis – papules BJD 133:671-678, 1995 Fiberglass dermatitis Kao Hsiung I Hseveh Ko Hseueh Tsa Chih 12:491-494, 1996 Iododerma Australas J Dermatol 29:179-180, 1988 Mudi-chood – due to oils applied to hair; papulosquamous eruption of nape of neck and upper back; begin as follicular pustules then brown-black papules with keratinous rim Int J Dermatol 31:396-397, 1992

Abscess of neck – Salmonella Head Neck 13:153-155, 1991 Actinomycosis, cervicofacial – midline cervical cleft with sinus tract Laryngoscope 94:1198-1217, 1984

AIDS – photosensitivity Anthrax – eschar of neck Cutis 67:488-492, 2001; J Appl Microbiol 87:303, 1999; Clin Inf Dis 19:1009-1014, 1994; Cutis 48:113-114, 1991; Cutis 40:117-118, 1987 Bacterial adenitis Brucellosis Candidiasis Carbuncle Cat scratch disease Ped Clin North Am 6:1151-1160, 1993 Chromomycosis AD 113:1027-1032, 1997; BJD 96:454-458, 1977; AD 104:476-485, 1971 Coccidioidomycosis AD 1345:365-370, 1998 Cryptococcosis – supraclavicular mass Head and Neck 21:239-246, 1999 Demodicidosis – papular eruption in HIV patients of head and neck J Med Assoc Thai 74:116-119, 1991; JAAD 20:197-201, 1989 Dental sinus – midline cervical cleft with sinus tract J Derm Surg Oncol 7:981-984, 1981; JAAD 2:521-524, 1980 Eikenella corrodens – neck wound Clin Inf Dis 33:70-75, 2001 Herpes simplex Tyring p.83, 2002; eczema herpeticum (Kaposi’s varicelliform eruption) Clin Inf Dis 32:1480, 1500-1501, 2001; Rook p.1028, 1998, Sixth Edition; Arch Dis Child 60:338-343, 1985 Herpes zoster Histoplasmosis Impetigo Infectious eczematoid dermatitis Infectious mononucleosis Ped Clin North Am 6:1151-1160, 1993 Kaposi’s varicelliform eruption Leishmaniasis – nodule, crusting, ulceration, scarring Trans R Soc Trop Med Hyg 81:606, 1987; Cutis 38:198-199, 1986 Lemierre’s syndrome (human necrobacillosis) – Fusobacterium necrophorum; suppurative thrombophlebitis of tonsillar and peritonsillar veins and internal jugular vein; oropharyngeal pain, neck swelling, pulmonary symptoms, arthralgias Clin Inf Dis 31:524-532, 2000 Lymphadenitis Ped Clin North Am 6:1151, 1993 Lymphadenopathy, reactive – supraclavicular mass Head and Neck 21:239-246, 1999 Molluscum contagiosum Mycobacterium avium-intracellulare Ped Clin North Am 6:1151-1160, 1993 Mycobacterium fortuitum – following neck liposuction Dermatol Surg 26:588-590, 2000; red plaque mimicking lupus vulgaris BJD 147:170-173, 2002 Mycobacterium scrofulaceum Ped Clin North Am 6:1151-1160, 1993 Mycobacterium tuberculosis – scrofuloderma – infected lymph node, bone, joint, lacrimal gland with overlying red-blue nodule which breaks down, ulcerates, forms fistulae, scarring with adherent fibrous masses which may be fluctuant and draining Tyring p.327, 2002; BJD 134:350-352, 1996; Ped Clin North Am 6:1151-1160, 1993; scrofuloderma after BCG vaccination Ped Derm 19:323-325, 2002; J Dermatol 21:106-110, 1924; tuberculous cold abscess of the neck BJD 142:387-388, 2000 Nocardiosis North American blastomycosis Paracoccidioidomycosis Br J Radiol 72:717-722, 1999 Pasteurella multocida Pediculosis

Phaeoacremonium inflatipes – fungemia in child with aplastic anemia; swelling and necrosis of lips, periorbital edema, neck swelling Clin Inf Dis 40:1067-1068, 2005 Pityrosporum folliculitis Mycoses 40 (suppl 1):29-32, 1997 Plague Pyogenic lymphadenitis Rat-bite fever Roseola infantum (human herpesvirus 6) – rose-pink macules start on neck and trunk, then spread to face and extremities Rook p.998,1025, 1998, Sixth Edition Scabies, nodular Ped Derm 11:264-266, 1994 Sporotrichosis Staphylococcus aureus – folliculitis; furunculosis; staphylococcal lymphadenitis Head and Neck 21:239-246, 1999 Sycosis barbae Syphilis – primary chancre Rook p.1244, 1998, Sixth Edition secondary AD 133:1027, 1030, 1997 Tinea corporis Tinea versicolor Toxoplasmosis Tularemia Verruca vulgaris Verruga peruana Am J Trop Med and Hygiene 50:143, 1994

Amyloidosis – nodular amyloidoisis AD 139:1157-1159, 2003 Colloid milium Clin Exp Dermatol 18:347-350, 1993; BJD 125:80-81, 1991 Juvenile xanthogranuloma (generalized lichenoid juvenile xanthogranuloma) – face, neck, scalp, upper trunk BJD 126:66-70, 1992

Eosinophilic pustular folliculitis J Dermatol 25:178-184, 1998 Folliculitis nuchae scleroticans Hautarzt 39:739-742, 1988 Malignant pyoderma Pseudofolliculitis barbae Rosai-Dorfman disease – sinus histiocytosis with massive lymphadenopathy Sarcoid Laryngoscope 87:2038-2048, 1977 Toxic epidermal necrolysis

α1-anti-trypsin panniculitis Cryoglobulinemia – papules JAAD 25:21-27, 1991 Essential fatty acid deficiency Goiter Pellagra (niacin deficiency) – Casal’s necklace; red pigmented sharply marginated photodistributed rash, including druginduced pellagra-like dermatitis – 6-mercaptopurine, 5fluorouracil,INH (all of the above – also seb derm-like); resembles Hartnup disease Cutis 68:31-34, 2001; Ped Derm 16:95-102, 1999; BJD 125:71-72, 1991 Porphyria – porphyria cutanea tarda with calcinosis; congenital erythropoietic porphyria BJD 148:160-164, 2003 Pregnancy – hyperpigmentation of neck, nipples, anogenital skin Rook p.1780, 1998, Sixth Edition

Pretibial myxedema JAAD 46:723-726, 2002 Pruritic urticarial papules and plaques of pregnancy Z hautkr 65:831-832, 1990 Xanthomas, including plane xanthomatosis BJD 133:961-966, 1995

Acrochordon (skin tags) Actinic keratosis Adenoid cystic carcinoma JAAD 17:113-118, 1987 Angiomatoid fibrous histiocytoma Dermatol Surg 26:491-492, 2000 Apocrine carcinoma Cancer 71:375-381, 1993 Atypical fibroxanthoma Basal cell carcinoma Cancer 92:354-358, 2001; following radiotherapy AD 108:523-527, 1973 Basosquamous carcinoma Otolaryngol Head Neck Surg 87:420-427, 1979 Bowenoid papulosis – neck papules JAAD 41:867-870, 1999 Carotid body tumor Cephalic histiocytomas Am J Dermatopathol 15:581-586, 1993 Chondroid syringoma Ear Nose Throat J 75:104-108, 1996 Cylindromas – scalp, face, nose, around ears and neck Cysts on neck

Branchial cleft anomaly (cyst, sinus, and/or fistula) Bronchogenic cyst Dermoid cyst Epidermoid cyst Eruptive vellus hair cysts Heterotopic salivary gland tissue Milia Pilar cyst Steatocystoma multiplex/simplex Thyroglossal duct cyst Teratomas

Epidermal nevus Epidermoid inclusion cyst Eruptive fibromas J Cut Pathol 25 (2):122-125, 1998 Eruptive histiocytoma J Dermatol 20:105-108, 1993 Eruptive syringomas Eruptive vellus hair cysts Ped Derm 5:94-96, 1988 Ganglioneuroma of cervical sympathetic chain Ped Clin North Am 6:1151-1160, 1993 Giant cell fibroblastoma of soft tissue – neck and trunk Ped Derm 18:255-257, 2001 Heterotopic submandibular salivary glands – submental mass Ped Clin North Am 6:1151-1160, 1993 Hibernoma – neck, axilla, central back; vascular dilatation overlying lesion AD 73:149-157, 1956 Benign cephalic histiocytosis – neck papules AD 135:1267-1272, 1999 Inflammatory linear verrucous epidermal nevus (ILVEN) Infantile myofibromatosis – red to skin-colored nodules AD 134:625-630, 1998 Inverted follicular keratosis J Clin Pathol 28:465-471, 1975 Kaposi’s sarcoma Otolaryngol Head Neck Surg 111:618-624, 1994; Ann Intern Med 103:744-750, 1985 Keloids Rook p.2056-2057, 1998, Sixth Edition Keratoacanthoma, including Ferguson-Smith tumors Ann Dermatol Venereol 104:206-216, 1977

Leukemia cutis Laryngoscope 86:1856-1863, 1976; cervical adenopathy Ped Clin North Am 6:1151-1160, 1993 Lipofibromata, eruptive AD 119:612-614, 1983 Lipoma supraclavicular mass Head and Neck 21:239-246, 1999; Rook p.2431, 1998, Sixth Edition; spindle cell lipomas JAAD 48:82-85, 2003; large neck mass in infancy Lymphadenoma, cutaneous – papules or nodules of head and neck BJD 128:339-341, 1993 Lymphadenopathy, malignant Lymphoma – cutaneous T-cell lymphoma and Ki-1+ lymphoma; anaplastic large cell B-cell lymphoma; cutaneous B-cell lymphomas Am J Surg Pathol 10:454-463, 1986; angiocentric lymphoma – edema of face and neck Indian J Pathol Microbiol 34:293-295, 1991; Hodgkin’s disease – ulcerated papules and nodules AD 133:1454-1455, 1457-1458, 1997; Hodgkin’s disease mimicking scrofuloderma Dermatology 199:268-270, 1999; granulomatous slack skin syndrome (CTCL) BJD 142:353-357, 2000; Ped Derm 14:204-208, 1997; AD 121:250-252, 1985; lymphomatous lymphadenopathy – neck masses Ped Clin North Am 6:1151-1160, 1993; Burkitt’s lymphoma Tyring p.155, 2002 Lymphoplasmacytoid immunocytoma Hautarzt 44:172-175, 1993 Malignant proliferating trichilemmal tumor BJD 150:156-157, 2004 Melanocytic nevus Rook p.1722-1723, 1998, Sixth Edition Melanoma Acta Oncol 38:1069-1074, 1999; Am J Trop Med and Hygiene 50 (2):143, 1994 Metastases – supraclavicular masses; breast, uterine, cervical, lung, stomach, oropharyngeal carcinomas Head and Neck 21:239-246, 1999 Mucoepidermoid carcinoma Laryngoscope 93:464-467, 1983 Neurilemmoma Neuroblastoma of cervical sympathetic chain – neck mass Ped Clin North Am 6:1151-1160, 1993 Neurofibroma – plexiform neurofibromas of brachial plexus Ped Clin North Am 6:1151-1160, 1993 Neurothekoma Am J Surg Pathol 14:113-120, 1990 Nevi, melanocytic, including eruptive melanocytic nevi (papules) JAAD 37:337-339, 1997; J Dermatol 22:292-297, 1995 Nevus comedonicus Nevus sebaceus Paraganglioma – neck mass Ped Clin North Am 6:1151-1160, 1993 Pilomatrixoma Otolaryngol Head Neck Surg 125:510-515, 2001; J Cutan Pathol 18:20-27, 1991 Porokeratosis Rhabdomyosarcoma – neck nodule in children JAAD 31:871-876, 1994; Ped Clin North Am 6:1151-1160, 1993 Schwannoma – supraclavicular mass Head and Neck 21:239-246, 1999 Squamous cell carcinoma J Laryngol Otol 110:694-695, 1996 Syringocystadenoma papilliferum AD 121:1198-1201, 1985 Syringoma Thymus gland rests – mass of lower neck or suprasternal notch Ped Clin North Am 6:1151-1160, 1993 Thyroid adenoma, carcinoma, colloid cysts – neck nodules Ped Clin North Am 6:1151-1160, 1993 Verrucous carcinoma Tyring p.271, 2002 Warthin’s tumor, extraparotid – skin-colored neck nodule JAAD 40:468-470, 1999 Warty dyskeratoma Ghatan p.86, 2002, Second Edition

Acrokeratosis paraneoplastica (Bazex syndrome) J Laryg Otol 110:899-900, 1996 Necrobiotic xanthogranuloma with paraproteinemia Normolipemic plane xanthomatosis BJD 135:460-462, 1996

Actinic granuloma AD 122:43-47, 1986 Berloque dermatitis Cutis rhomboidalis nuchae Photoallergic contact dermatitis Phytophotodermatitis Poikiloderma of Civatte Ann Dermatol Syphilol 9:381-420, 1938 Riehl’s melanosis Ghatan p.86, 2002, Second Edition

Acanthosis nigricans/pseudoacanthosis nigricans Acne keloidalis nuchae Acne vulgaris AD 131:341-344, 1995 Alopecia mucinosa (follicular mucinosis) – neck plaque JAAD 38:622-624, 1998; Dermatology 197:178-180, 1998; JAAD 10:760-768, 1984; AD 76:419-426, 1957 Atopic dermatitis Anetoderma of Jadassohn AD 120:1032-1039, 1984 Centrifugal lipodystrophy Dermatology 188:142-144, 1994 Confluent and reticulated papillomatosis Cutis laxa Darier’s disease Dermatology 188:157-159, 1994 Diaper dermatitis with rapid dissemination – expanding nummular dermatitis of trunk, and red scaly plaques of neck and axillae (‘psoriasiform id’) BJD 78:289-296, 1966 Dowling-Degos disease Elastoderma JAAD 53:S147-149, 2005 Elastosis perforans serpiginosa J Dermatol 24:458-465, 1997; Hautarzt 43:640-644, 1992; AD 97:381-393, 1968 Epidermolysis bullosa – recessive inverse dystrophic – groin, axillae, neck, lower back, nail dystrophy, oral erosions (dermolytic dystrophic) AD 124:544-547, 1988 Epidermolytic hyperkeratosis Erythema annulare centrifugum Erythema of Jacquet Fibroelastolytic papulosis of the neck BJD 173:461-466, 1997 Granuloma annulare Citos 55:158-160, 1995 Grover’s disease (benign papular acantholytic dermatosis) AD 112:814-821, 1976 Hailey-Hailey disease BJD 126:275-282, 1992; Arch Dermatol Syphilol 39:679-685, 1939 Ichthyosis vulgaris Impetigo herpetiformis Juxtaclavicular beaded lines J Cutan Pathol 18:464-468, 1991 Kimura’s disease Am J Kid Dis 11:353-356, 1988 Lichen nitidus Lichen planus Lichen sclerosus et atrophicus, guttate Lichen simplex chronicus Rook p.668, 1998, Sixth Edition

Lichen spinulosus JAAD 22:261-264, 1990 Miliaria rubra in infants BJD 99:117-137, 1978 Napkin psoriasis Pityriasis rosea Pityriasis rubra pilaris Prurigo nodularis Pseudofolliculitis barbae Psoriasis Scleredema of Buschke (pseudoscleroderma) JAAD 11:128-134, 1984 Seborrhiasis Syringolymphoid hyperplasia JAAD 49:1177-1180, 2003 Terra firme of teens – anterior neck Upper dermal elastolysis J Cutan Pathol 21:533-540, 1994 Vitiligo White fibrous papulosis of the neck Int J Derm 35:720-722, 1996; BJD 127:295-296, 1992 X-linked ichthyosis

Factitial dermatitis

Acrogeria Dermatology 192:264-268, 1996 Becker’s syndrome – discrete or confluent brown macules of neck, forearms AD Syphilol 40:987-998, 1939 Birt-Hogg-Dube syndrome AD 133:1163-1166, 1997 Branchio-oculo-facial syndrome (pseudocleft of upper lip, cleft-lip-palate, and hemangiomatous branchial cleft) Am J Med Genet 27:943-951, 1987 Branchio-oto-renal syndrome (Melnick-Fraser syndrome) – autosomal dominant, chromosome 8q – abnormal pinna, prehelical pits, renal anomalies, branchial cleft fistulae and/or cysts pre-auricular sinus tract or cyst Am J Nephrol2:144-146, 1982; Am J Med Genet 2:241-252, 1978; Clin Genet 9:23-34, 1976 Buschke-Ollendorff syndrome Eur J Dermatol 11:576-579, 2001 Coffin-Siris syndrome – webbed neck, bifid scrotum, umbilical and inguinal hernias JAAD 46:161-183, 2002 Costello syndrome – warty papules around nose and mouth, legs, perianal skin; loose skin of neck, hands, and feet, thick, redundant palmoplantar surfaces, hypoplastic nails, short stature, craniofacial abnormalities Eur J Dermatol 11:453-457, 2001; Am J Med Genet 82:187-193, 1999; JAAD 32:904-907, 1995; Am J Med Genet 47:176-183, 1993; Aust Paediat J 13:114-118, 1977 Cowden’s disease Craniocarpotarsal dysplasia (whistling face syndrome) – webbed neck Birth Defects 11:161-168, 1975 Diffuse pigmentation of trunk and neck with subsequent white macules Proc R Soc Med 48:179-180, 1955 Diffuse pigmentation with macular depigmentation of trunk with reticulate pigmentation of neck Hautarzt 6:458-460, 1955 Distichiasis and lymphedema – webbed neck Dowling-Degos disease Down’s syndrome – webbed neck Dyskeratosis congenita (Zinsser-Engman-Cole syndrome) – Xq28 J Med Genet 33:993-995, 1996; Dermatol Clin 13:33-39, 1995; BJD 105:321-325, 1981

Ekbom’s syndrome (myoclonic epilepsy and ragged muscle fibers) (mitochondrial syndrome) – cervical lipomas JAAD 39:819-823, 1998 Encephalocraniocutaneous lipomatosis Ped Derm 10:164-168, 1993 Epidermodysplasia verruciformis Franceschetti-Jadassohn-Naegeli syndrome – generalized reticulated hyperpigmentation, accentuated in neck and axillae JAAD 10:1-16, 1984 Gardner’s syndrome – nuchal-type fibroma Am J Surg Pathol 24:1563-1567, 2000 Goldenhaar syndrome (oculoauriculovertebral syndrome) – epibulbar dermoid cysts, vertebral defects, accessory tragi Int J Derm 19:479-486, 1980 Hidrotic ectodermal dysplasia Hautarzt 42:645-647, 1991 Hunter’s syndrome – skin-colored to white papules of nape of neck AD 113:602-605, 1977 Infantile myofibromatosis Int J Pediatr Otorhinolaryngol 51:181-186, 1999; Ped Derm 8:306-309, 1991; Ped Derm 5:37-46, 1988 Juvenile hyaline fibromatosis (systemic hyalinosis) – translucent papules or nodules of scalp, face, neck, trunk, gingival hypertrophy, flexion contractures of large and small joints JAAD 16:881-883, 1987 Kawasaki’s disease – cervical adenopathy Ped Clin North Am 6:1151-1160, 1993 Klippel-Feil anomaly – webbed neck J Bone Jt Surg 56:1246-1253, 1974 LEOPARD (Moynahan’s) syndrome – CALMs, granular cell myoblastomas, steatocystoma multiplex, small penis, hyperelastic skin, low-set ears, short webbed neck, short stature, syndactyly JAAD 46:161-183, 2002; Am J Med 60:447-456, 1976; JAAD 40:877-890, 1999; J Dermatol 25:341-343, 1998; Am J Med 60:447-456, 1976; AD 107:259-261, 1973 Lymphedema-distichiasis syndrome – periorbital edema, vertebral abnormalities, spinal arachnoid cysts, congenital heart disease, thoracic duct abnormalities, hemangiomas, cleft palate, microphthalmia, strabismus, ptosis, short stature, webbed neck Ped Derm 19:139-141, 2002 Madelung’s deformity McCune-Albright syndrome – café au lait macule Ped Derm 8:35-39, 1991 Menkes’ kinky hair syndrome – silvery hair, generalized hypopigmentation, lax skin of brows, neck, and thighs Ped Derm 15:137-139, 1998 Microphthalmia with linear skin defects syndrome (MLS syndrome) (microphthalmia, dermal aplasia, and sclerocornea (MIDAS) syndrome) – X-linked dominant; atrophic Blaschko linear scars of face and neck; linear red atrophic skin (resembles aplasia cutis) Textbook of Neonatal Dermatology, p.466-467, 2001; Am J Med Genet 49:229-234, 1994 Mottled pigmentation of neck and elbows Z Haut-u Geschl Krankh 32:33-44, 1962 Multiple endocrine neoplasia syndrome type II – medullary carcinoma of the thyroid Ped Clin North Am 6:1151-1160, 1993 Multiple pterygium syndrome – webbed neck J Med Genet 24:733-749, 1987 Neu-Laxova syndrome – short neck; mild scaling to harlequin ichthyosis appearance; ichythosiform scaling, increased subcutaneous fat and atrophic musculature, generalized edema and mildly edematous feet and hands, absent nails; microcephaly, intrauterine growth retardation, limb contractures, low-set ears, sloping forehead; small genitalia, eyelid and lip

closures, syndactyly, cleft lip and palate, micrognathia; autosomal recessive; uniformly fatal Ped Derm 20:25-27,78-80, 2003; Curr Prob Derm 14:71-116, 2002; Clin Dysmorphol 6:323-328, 1997; Am J Med Genet 35:55-59, 1990 Neurofibromatosis – type I; segmental neurofibromatosis J La State Med Soc 146:183-186, 1994 Noonan’s syndrome – webbed neck, short stature, malformed ears, nevi, keloids, transient lymphedema, ulerythema ophyrogenes, keratosis follicularis spinulosa decalvans JAAD 46:161-183, 2002; J Med Genet 24:9-13, 1987 Occipital horn syndrome (Ehlers-Danlos syndrome, type IX) – long neck Am J Hum Genet 41:A49, 1987 Oculo-auricular vertebral syndrome – short neck, epibulbar dermoid tumors, abnormal hair Ped Derm 20:182-184, 2003 Patau’s syndrome (trisomy 13) – loose skin of posterior neck, parieto-occipital scalp defects, abnormal helices, low-set ears, simian crease of hand, hyperconvex narrow nails, polydactyly Ped Derm 22:270-275, 2005; Rook p.3016, 1998, Sixth Edition Pseudoxanthoma elasticum – linear and reticulated cobblestoned yellow papules and plaques AD 124:1559, 1988; JAAD 42:324-328, 2000; Dermatology 199:3-7, 1999; PXE and acrosclerosis Proc Roy Soc Med 70:567-570, 1977 Short stature, mental retardation, facial dysmorphism, short webbed neck, skin changes, congenital heart disease – xerosis, dermatitis, low-set ears, umbilical hernia Clin Dysmorphol 5:321-327, 1996 Sjögren-Larsson syndrome – verrucous hyperkeratosis of flexures, neck, and periumbilical folds; mental retardation, spastic diplegia, short stature, kyphoscoliosis, retinal changes, yellow pigmentation, intertrigo – deficiency of fatty aldehyde dehydrogenase Chem Biol Interact 130-132:297-307, 2001; Am J Hum Genet 65:1547-1560, 1999; JAAD 35:678-684, 1996 Steatocystoma multiplex Sweet’s syndrome – red plaque with or without bullae or pustules JAAD 40:838-841, 1999; AD 134:625-630, 1998; Eur J Gastro Heptl 9:715-720, 1997; JAAD 31:535-536, 1994; BJD 76:349-356, 1964 Thrombocytopenia-absent radius syndrome (TAR syndrome) – cutis laxa of neck; congenital thrombocytopenia, bilateral absent or hypoplastic radii, port wine stain of head and neck AD 126:1520-1521, 1990; Am J Pediatr Hematol Oncol 10:51-64, 1988 Trisomy 13 (Patau syndrome) – redundant skin of neck J Genet Hum 23:83-109, 1975 Trisomy 18 syndrome – redundant skin of neck J Med Genet 15:48-60, 1978 Turner’s syndrome (XO in 50%) – webbed neck, lymphedema of neck; low posterior hairline, low misshapen ears, peripheral edema at birth which resolves by age 2; redundant neck skin in newborn; small stature, broad shield-shaped cest with widely spaced nipples, arms show wide carrying angle, high arched palate, cutis laxa of neck and buttocks, short fourth and fifth metacarpals and metatarsals, hypoplastic nails, keloid formation, increased numbers of nevi; skeletal, cardiovascular, ocular abnormalities; increased pituitary gonadotropins with low estrogen levels JAAD 50:767-776, 2004; JAAD 46:161-183, 2002; JAAD 40:877-890, 1999; NEJM 335:1749-1754, 1996; halo nevi of the neck JAAD 51:354-358, 2004 Weaver-Williams syndrome (cleft palate, microcephaly, mental retardation, musculoskeletal mass deficiency) – long neck Birth Defects 13:69-84, 1977 49,XXXXY syndrome – webbed neck Syndromes of the Head and Neck, p.59-60, 1990

Dental treatment – soft tissue cervicofacial emphysema after dental treatment AD 141:1437-1440, 2005 Fiddler’s neck – lichenification with erythema, hyperpigmentation, papules, pustules, and cysts BJD 98:669-674, 1978 Hickey (passion mark) Strangulation purpura Subcutaneous emphysema AD 134:557-559, 1998

Acquired elastotic hemangioma – red plaque with vascular appearance JAAD 47:371-376, 2002 Aneurysmal dilatation of the internal jugular vein – soft blue neck mass Ped Clin North Am 6:1151-1160, 1993 Angiosarcoma BJD 138:692-694, 1998; Australas Radiol 39:277-281, 1995; Cancer 44:1106-1113, 1979 Angioma serpiginosum Arteriovenous fistulae – congenital or acquired; red pulsating nodules with overlying telangiectasia – extremities, head, neck, trunk Rook p.2237, 1998, Sixth Edition Cystic hygroma (lymphatic malformation) Ped Clin North Am 6:1151-1160, 1993; NEJM 309:822-825, 1983 Dabska tumor (malignant endovascular papillary endothelioma) – head and neck of infants JAAD 49:887-896, 2003 Hemangioma – symptomatic hemangiomas of the airway with airway obstruction and cutaneous hemangiomas in a ‘beard’ distribution J Pediatr 131:643-646, 1997; large neck mass Non-involuting congenital hemangioma JAAD 53:185-186, 2005 Lymphangioma Pseudo-Kaposi’s sarcoma Salmon patch (nevus simplex) (‘stork bite’) – pink macules with fine telangiectasias of the nape of the neck, glabella, forehead, upper eyelids, tip of nose, upper lip, midline lumbosacral area Ped Derm 73:31-33, 1983 Tufted angioma – dull red, purple, or red-brown JAAD 49:887-896, 2003; Ped Derm 19:388-393, 2002 Unilateral nevoid telangiectasia Venous lakes

Dermatitis herpetiformis Graft vs. host reaction, acute Taiwan I Hsueh Hui Tsa Chih 88:657-662, 1989 Lupus erythematosus – papulonodular mucinosis; papules of neck JAAD 32:199-205, 1995; AD 114:432-435, 1978 Urticaria

Accessory tragus – facial, glabellar papule – isolated, Treacher Collins syndrome (mandibulofacial dysostosis; autosomal dominant), Goldenhaar syndrome ((oculo-auriculo-vertebral syndrome) – macroglossia, preauricular tags, abnormal pinnae,

facial asymmetry, macrostomia, epibulbar dermoids, facial weakness, central nervous system, renal, and skeletal anomalies), Nagers syndrome, Wolf-Hirschhorn syndrome (chromosome 4 deletion syndrome), oculocerebrocutaneous syndrome Ped Derm 17:391-394, 2000; Townes-Brocks syndrome Am J Med Genet 18:147-152, 1984; VACTERL syndrome J Pediatr 93:270-273, 1978 Branchial remnants – branchial cleft cyst/sinus Plastic and Reconstructive Surgery 100:32-39, 1997 Cervical accessory auricle (wattle) Cervical braid Brit J Plast Surg 43:369-370, 1990 Cutaneous cartilaginous rest Textbook of Neonatal Dermatology, p.118-119, 2001; AD 121:22-23, 1985; in oculo-auricular-vertebral spectrum Syndromes of the Head and Neck; Oxford Monographs on Medical Genetics No. 19; Oxford University Press; Oxford/New York; 1990 Occult spinal dysraphism Ped Derm 12:256-259, 1995 Rhabdomyomatous mesenchymal hamartoma – pedunculated papule associated with a midline cervical cleft AD 141:1161-1166, 2005 Supernumerary nipples Cutis 71:344-346, 2003 Thyroglossal duct cyst

Cyclosporine – pseudofolliculitis barbae-like lesions Dermatologica 172:24-30, 1986

Chrysiasis BJD 133:671-678, 1995 Fiberglass dermatitis Kao Hsiung I Hseveh Ko Hseueh Tsa Chih 12:491-494, 1996

Candidiasis Chromomycosis – feet, legs, arms, face, and neck AD 113:1027-1032, 1997; BJD 96:454-458, 1977; AD 104:476-485, 1971 Coccidioidomycosis Acta Cytologica 38:422-426, 1994 Demodicidosis J Med Assoc Thai 74:116-119, 1991 Dental sinus Mycobacterium tuberculosis – after BCG vaccination J Derm 21:106-110, 1924 Myiasis – cuterebrid myiasis; neck nodules Ped Derm 21:515-516, 2004 Pediculosis – head lice – pruritic papules of nape of neck Rook p.1441, 1998, Sixth Edition; generalized pruritic eruption NEJM 234:665-666, 1946 Pityrosporum folliculitis Mycoses 40 (supp 1) 29-32, 1997 Scabies Ped Derm 11:264-266, 1994 Staphylococcus aureus – folliculitis Sycosis barbae Tinea barbae Verruca vulgaris Verruga peruana Am J Trop Med and Hygiene 50:143, 1994

Benign cephalic histiocytosis – cheeks, forehead, earlobes, neck JAAD 47:908-913, 2002; Ped Derm 11:265-267,

1994; Ped Derm 6:198-201, 1989; AD 122:1038-43, 1986; JAAD 13:383-404, 1985 Lichen myxedematosis Rook p.2626-2617, 1998, Sixth Edition; JAAD 33:37-43, 1995; discrete papular lichen myxedematosus Cutis 75:105-112, 2005 Plane xanthomatosis BJD 133:961-966, 1995 Self-healing (papular) juvenile cutaneous mucinosis – arthralgias Ped Derm 20:35-39, 2003; JAAD 44:273-281, 2001; Ped Derm 14:460-462, 1997; AD 131:459-461, 1995; JAAD 11:327-332, 1984; Ann DV 107:51-57, 1980; of adult JAAD 50:121-123, 2004, BJD 143:650-651, 2000; Dermatology 192:268-270, 1996

Eosinophilic pustular folliculitis J Dermatol 25:178-184 Erythema nodosum Rook p.2200, 1998, Sixth Edition Folliculitis nuchae scleroticans Hautarzt 39:739-742, 1988 Kimura’s disease Am J Kidney disease 11:353-356, 1988 Sarcoid Rook p.2687, 1998, Sixth Edition; AD 133:882-888, 1997; NEJM 336:1224-1234, 1997; Clinics in Chest Medicine 18:663-679, 1997

Cryoglobulinemia JAAD 25:21-27, 1991 Pruritic urticarial papules and plaques of pregnancy (PUPPP) Z Hautkr 65:831-832, 1990

Acrochordon (skin tag) Rook p.1661, 1998, Sixth Edition Adenoid cystic carcinoma JAAD 17:113-118, 1987 Angiosarcoma BJD 138:692-694, 1998 Apocrine carcinoma Cancer 71:375-381, 1993 Atypical fibroxanthoma Sem Cut Med Surg 21:159-165, 2002; Cutis 51:47-48, 1993; Cancer 31:1541-1552, 1973 Basal cell carcinoma Rook p.1681-1683, 1998, Sixth Edition; Acta Pathol Mibrobiol Scand 88A:5-9, 1980 Basaloid carcinoma of lung – metastasis JAAD 49:523-526, 2003 Cephalic histiocytomas Am J Dermatopathol 15:581-586, 1993 Cutaneous lymphadenoma – papules or nodules of head and neck BJD 128:339-341, 1993 Cylindromas BJD 151:1084-1086, 2004 Dermal duct tumor – red nodule of neck AD 140:609-614, 2004 Dermatomyofibroma – oval nodule or firm plaque Clin Exp Dermatol 21:307-309, 1996 Desmoplastic trichoepithelioma AD 138:1091-1096, 2002; AD 132:1239-1240, 1996; Cancer 40:2979-2986, 1977 Encephalocraniocutaneous lipomatosis Ped Derm 10:164-168, 1993 Eruptive fibromas J Cutan Pathol 25:122-125 Eruptive histiocytoma J Dermatol 20:105-108, 1993 Eruptive lipofibromas AD 119:612-614, 1983 Eruptive vellus hair cysts Fibrosarcoma, neonatal JAAD 50:S23-25, 2004 Fibrous hamartoma of infancy – neck nodule Ped Dev Pathol 2:236-243, 1999

Hamartoma moniliformis – linear array of skin-colored papules of face and neck AD 101:191-205, 1970 Infantile myofibromatosis – single or multiple; head, neck, trunk JAAD 41:508, 1999; AD 134:625-630, 1998 Inverted follicular keratosis J Clin Pathol 28:465-471, 1975 Kaposi’s sarcoma Ann Intern Med 103:744-750, 1985 Keratoacanthomas of Ferguson-Smith – multiple self-healing keratoacanthomas JAAD 49:741-746, 2003; Ann DV 104:206-216, 1977; BJD 46:267-272, 1934 Leiomyomas – grouped, linear or dermatomal JAAD 52:410-416, 2005; JAAD 46:477-490, 2002 Leiomyosarcoma – blue-black; also red, brown, yellow or hypopigmented JAAD 46:477-490, 2002 Leukemia – chronic lymphocytic leukemia Laryngoscope 86:1856-1863, 1976 Lymphoepithelioma-like carcinoma Mod Pathol 1:359-365, 1988 Lymphoma – CTCL; Ki-1+ lymphoma; Hodgkin’s disease AD 133:1454-1455, 1457-1458, 1997; B-cell lymphoma JAAD 53:479-484, 2005; xanthomatous infiltration of the neck Eur J Derm 10:481-483, 2000 Lymphoplasmocytoid immunocytoma Hautarzt 44:172-175, 1995 Melanocytic nevus Rook p.1722-1723, 1998, Sixth Edition; eruptive melanocytic nevi JAAD 37:337-339, 1997; J Dermatol 22:292-297, 1995 Melanoma Merkel cell carcinoma – pink to violaceous nodule Sem Cut Med Surg 21:159-165, 2002 Milia Acta Derm Venereol 71:334-336, 1991 Mucinous carcinoma JAAD 52:S76-80, 2005 Multiple myeloma AD 139:475-486, 2003 Myofibroma J Cutan Pathol 23:445-457, 1996; Histopathol 22:335-341, 1993 Neurilemmoma (schwannoma) – pink-gray or yellowish nodules of head and neck Rook p.2363, 1998, Sixth Edition Neurofibroma Neuroma – palisaded encapsulated neuroma AD 140:1003-1008, 2004 Neurothekoma Am J Surg Pathol 14:113-120, 1990 Pilomatrixoma J Cutan Pathol 18:20-27, 1991 Pilomatrix carcinoma – multiple of head and neck Otolaryngol Head Neck Surg 109:543-547, 1993 Porokeratotic eccrine ostial and dermal duct nevus (linear eccrine nevus with comedones) AD 138:1309-1314, 2002 Rhabdomyosarcoma JAAD 31:871-876, 1994 Solitary fibrous tumor of the skin – facial, scalp, posterior neck nodule JAAD 46:S37-40, 2002 Squamous cell carcinoma Syringocystadenoma papilliferum AD 121:1198-1201, 1985; linear verrucous papules AD 138:1091-1096, 2002 Syringomas, eruptive Warty dyskeratoma – face, neck, scalp, axillae Ghatan p.341, 2002, Second Edition

Acrokeratosis paraneoplastica (Bazex syndrome) J Laryng Otol 110:899-900, 1996

Actinic granuloma AD 122:43-47, 1986

Acantholytic dermatosis Nippon Hifuka Gakkai Zasshi 10:453-460, 1991 Acne vulgaris AD 131:341-344, 1995 Alopecia mucinosa (follicular mucinosis) – neck papules JAAD 38:622-624, 1998; Dermatology 197:178-180, 1998; JAAD 10:760-768, 1984; AD 76:419-426, 1957 Anetoderma of Jadassohn Benign papular acantholytic dermatosis AD 112:814-821, 1976 Darier’s disease Dermatology 188:157-159, 1994 Disseminated and recurrent infundibulofolliculitis – neck, trunk, extremities J Derm 25:51-53, 1998; Dermatol Clin 6:353-362, 1988; AD 105:580-583, 1972 Elastosis perforans serpiginosa J Dermatol 24:458-465, 1997 Fibroelastolytic papulosis of the neck BJD 173:461-466, 1997 Granuloma annulare Cutis 55:158-160, 1995 Granuloma faciale, extrafacial BJD 145:360-362, 2001 Juxtaclavicular beaded lines J Cutan Pathol 18:464-468, 1991 Lichen sclerosus et atrophicus, guttate Lichen spinulosus JAAD 22:261-264, 1990 Psoriasis Upper dermal elastolysis J Cutan Pathol 21:533-540, 1994 White fibrous papulosis of the neck (fibroelastolytic papulosis) – cobblestoning with 2-4 mm skin-colored papules JAAD 51:958-964, 2004; Int J Derm 35:720-722, 1996; JAAD 20:1073-1077, 1989

Acrogeria Dermatol 192:264-268, 1996 Atrichia with keratin cysts – face, neck, scalp; then trunk and extremities Ann DV 121:802-804, 1994 Basaloid follicular hamartoma syndrome (generalized basaloid follicular hamartoma syndrome) – multiple skin-colored, red, and hyperpigmented papules of the face, neck chest, back, proximal extremities, and eyelids; syndrome includes milia-like cysts, comedones, sparse scalp hair, palmar pits, and parallel bands of papules of the neck (zebra stripes) JAAD 49:698-705, 2003; BJD 146:1068-1070, 2002; JAAD 43:189-206, 2000 Behçet’s syndrome – erythema nodosum; nodule AD 138:467-471, 2002 Birt-Hogg-Dube syndrome AD 133:1163-1166, 1997 Down’s syndrome – deep folliculitis of posterior neck Ghatan p.242, 2002, Second Edition Fibroblastic rheumatism – symmetrical polyarthritis, nodules over joints and on palms, elbows, knees, ears, neck, Raynaud’s phenomenon, sclerodactyly; skin lesions resolve spontaneously AD 131:710-712, 1995 Hunter’s syndrome – reticulated 2-10mm skin colored papules over scapulae, chest, neck, arms; X-linked recessive; MPS type II; iduronate-2 sulfatase deficiency; lysosomal accumulation of heparin sulfate and dermatan sulfate; short stature, full lips, coarse facies, macroglossia, clear corneas (unlike Hurler’s syndrome), progressive neurodegeneration, communicating hydrocephalus, valvular and ischemic heart disease, lower respiratory tract infections, adenotonsillar hypertrophy, otitis media, obstructive sleep apnea, diarrhea, hepatosplenomegaly, skeletal deformities (dysostosis multiplex), widely spaced teeth, dolichocephaly, deafness, retinal degeneration, inguinal and umbilical hernias Ped Derm 21:679-681, 2004; AD 113:602-605, 1977 Hypohidrotic ectodermal dysplasia Hautarzt 42:645-647, 1991

Juvenile hyaline fibromatosis (systemic hyalinosis) – translucent papules or nodules of scalp, face, neck, trunk, gingival hypertrophy, flexion contractures of large and small joints Ped Derm 21:154-159, 2004; JAAD 16:881-883, 1987 Lipoid proteinosis BJD 151:413-423, 2004; JID 120:345-350, 2003; Hum Molec Genet 11:833-840, 2002 Nevoid basal cell carcinoma syndrome JAAD 42:939-969, 2000; basal cell carcinomas – skin tag-like lesions in children with nevoid basal cell carcinoma syndrome JAAD 44:789-794, 2001 Pseudoxanthoma elasticum – linear and reticulated cobblestoned yellow papules and plaques JAAD 42:324-328, 2000; Dermatology 199:3-7, 1999; AD 124:1559, 1988; PXE and acrosclerosis Proc Roy Soc Med 70:567-570, 1977 Steatocystoma multiplex Sweet’s syndrome Eur J Gastro Hepatol 9:715-720, 1997 Tuberous sclerosis – angiofibromas

Fiddler’s neck BJD 98:669-674, 1978 Pseudofolliculitis barbae Dermatol Clin 6:387-395, 1988

Glomus tumor Rook p.2357, 1998, Sixth Edition Pseudo-Kaposi’s sarcoma overlying arteriovenous anastomosis following carotid endarterectomy Tufted angioma – deep red papule, plaque, or nodule of back or neck JAAD 52:616-622, 2005 Venous lakes

Allergic contact dermatitis – poison ivy or oak NEJM 352:700-707, 2005 Anti-centromere antibodies – ulcers and gangrene of the extremities Br J Rheumatol 36:889-893, 1997 Arthus reaction – necrosis of skin over deltoid muscle due to hepatitis B virus vaccine Clin Inf Dis 33:906-908, 2001; erythema, edema, hemorrhage, occasional necrosis Rook p.3364, 1998, Sixth Edition Bowel arthritis dermatitis syndrome – necrotic papules AD 135:1409-1414, 1999; Cutis 63:17-20, 1999; JAAD 14:792-796, 1986; Mayo Clin Proc 59:43-46, 1984; AD 115:837-839, 1979 Chronic granulomatous disease – necrotic ulcers; bacterial abscesses, perianal abscesses JAAD 36:899-907, 1997; AD 130:105-110, 1994; NEJM 317:687-694, 1987; AD 103:351-357, 1971 Congenital deficiency of leucocyte-adherence glycoproteins (CD11a (LFA-1), CD11b, CD11c, CD18) (leukocyte adhesion deficiency syndrome) – necrotic cutaneous abscesses, psoriasiform dermatitis, gingivitis, periodontitis, septicemia, ulcerative stomatitis, pharyngitis, otitis, pneumonia, peritonitis BJD 123:395-401, 1990 Connective tissue disease – eosinophilic vasculitis in connective tissue diseases; digital microinfarcts JAAD 35:173-182, 1996 Dermatomyositis – epidermal necrosis associated with internal malignancy Cutis 61:190-194, 1998

Graft vs. host disease, acute – epidermal necrosis AD 134:602-612, 1998 Hypersensitivity angiitis AD 138:1296-1298, 2002 Intrauterine blood transfusion – Rh incompatibility Linear IgA disease (TEN) JAAD 31:797-799, 1994 Lupus erythematosus – systemic – necrosis of the proximal nail fold Rook p.2474, 1998, Sixth Edition; epidermal necrosis mimicking toxic epidermal necrosis Clin Rheum Dis 8:207, 1982; vasculitis; gangrene of extremity due to vasculitis or thrombosis (anti-phospholipid antibodies) J Rheumatol 13:740-747, 1986; infarcts of fingertips JAAD 48:311-340, 2003; toxic epidermal necrolysis JAAD 48:525-529, 2003 Mixed connective tissue disease Rook p.2545, 1998, Sixth Edition; Am J Med 52:148-159, 1972 Myeloperoxidase deficiency – lip infection and necrosis Ped Derm 20:519-523, 2003 Pemphigus vegetans NEJM 352:700-707, 2005 Rheumatoid vasculitis – purpuric infarcts of paronychial areas (Bywater’s lesions) JAAD 53:191-209, 2005; BJD 77:207-210, 1965; bullae of fingertips and toetips with purpura Rook p.2184, 1998, Sixth Edition; BJD 77:207-210, 1965; large hemorrhagic lesions, gangrene with necrotizing arteritis Rook p.2214, 1998, Sixth Edition; peripheral gangrene JAAD 53:191-209, 2005; mononeuritis multiplex; papulonecrotic lesions JAAD 48:311-340, 2003 Severe combined immunodeficiency – necrotic bacterial skin infections resembling ecthyma gangrenosum

Gangrene of abdominal wall – due to intrauterine red-cell transfusion Am J Dis Child 117:593-596, 1969 Intrauterine epidermal necrosis JAAD 38:712-715, 1998 Subcutaneous fat necrosis of the newborn – necrotic bulla Ped Derm 20:257-261, 2003 Umbilical artery catheterization – legs, lumbar, buttocks (perinatal gangrene of the buttock) Textbook of Neonatal Dermatology, p.108,151, 2001; gangrene of legs AD 113:61-63, 1977; unilateral necrosis of buttock Arch Dis Child 55:815-817, 1980; JAAD 3:596-598, 1980

Actinomycin D extravasation Adriamycin – IV J Surg Res 75:61-65, 1998; Cancer Treat Rep 63:1003-1004, 1979; Cancer 38:1087-1094, 1976 Amiodarone Amitryptiline Burns 25:768-770, 1999 Arsenic – endothelial damage Beta blockers BJD 143:1356-1358, 2000 Bleomycin – gangrene Eur J Dermatol 8:221, 1998; AD 107:553-555, 1973 Buprenorphine – intra-arterial injection AD 138:1296-1298, 2002 Calcium gluconate infusion – IV JAAD 6:392-395, 1982 Chemotherapy injection site BJD 143:1356-1358, 2000 Cisplatin Cancer Treat Rep 67:199, 1983 IV cocaine JAAD 16:462-468, 1987 Compazine – intramuscular with infarction Coumarin necrosis – eschar and ulceration; associations include acquired protein C deficiency in chronic liver disease, chronic renal failure and dialysis, acute leukemia, systemic lupus erythematosus, anti-phospholipid antibody syndrome,

Epstein-Barr virus infection, acute respiratory distress syndrome, acute intravascular coagulation, plasmapheresis BJD 151:502-504, 2004; JAAD 47:766-769, 2002; Br J Surg 87:266-272, 2000; Thromb Haemost 78:785-790, 1997; Hematol Oncol Clin North Am 7:1291-1300, 1993; AD 128:105, 108, 1992; AD 123:1701a-1706a, 1987; with mutation of prothrombin gene NEJM 340:735, 1999; with familial type II protein C deficiency Am J Hematol 29:226-229, 1988; with acquired protein C deficiency Intensive Care Med 27:1555, 2001; with protein S deficiency Haemostasis 28:25-30, 1998; with Factor V Leiden and protein S deficiency Clin Lab Haematol 23:261-264, 2001; intravascular coagulation necrosis JAAD 25:882-888, 1991; late onset coumarin warfarin necrosis Am J Hematol 57:233-237, 1998 Cyclophosphamide BJD 143:1356-1358, 2000 Daunorubicin extravasation Oncol Nurs Forum 25:67-70, 1998 Depo-Provera – intramuscular BJD 143:1356-1358, 2000 Dextran injections – intramuscular AD 124:1722-1723, 1988 Doxorubicin hydrochloride Pharmazie 48:772-775, 1993 DPT vaccination site – embolia cutis medicamentosa (Nicolau syndrome) Actas Dermosifiliogr 95:133-134, 2004 Calcium salts – IV Dextrose – IV Dopamine – IV Ergot poisoning Rook p.2231, 1998, Sixth Edition Enoxaparin Ann Intern Med 125:521-522, 1996 Epidural blockade – necrosis of lower extremities Can J Anaesth 35:628-630, 1988 Epinephrine-containing cream Br J Clin Pract 38:191, 1984 Fluorescein extravasation Retina 7:89-93, 1987 5-fluorouracil – epidermal necrosis of psoriatic plaques following intravenous 5-FU BJD 147:824-825, 2002 Flunitrazepam abuse Acta DV 79:171, 1999 G-CSF – necrotizing vasculitis Ped Derm 17:205-207, 2000 Heparin necrosis Br J Haematol 111:992, 2000; Ann R Coll Surg Engl 81:266-269, 1999; JAAD 37:854-858, 1997; NEJM 336:588-589, 1997; Nephron 68:133-137, 1994; low molecular weight heparin Ann Haematol 77:127-130, 1998; at injection site Dermatology 196:264-265, 1998; Thromb Haemost 78:785-790, 1997; Australas J Dermatol 36:201-203, 1995; eschar and ulceration JAAD 47:766-769, 2002 Hydantoin (Dilantin) extravasation Hypertonic saline extravasation J Derm Surg Oncol 19:641-646, 1993 Infusion leakage in the newborn Br J Plast Surg 54:396-399, 2001 Interferon-α injection site JAAD 37:118-120, 1997; pegylated interferon alpha JAAD 53:62-66, 2005 Interferon-β JAAD 37:553-558, 1997; Dermatology 195:52-53, 1997 Interleukin-2 JAAD 29:66-70, 1993 Interleukin-3 BJD 143:1356-1358, 2000 Intra-articular anesthesia BJD 143:1356-1358, 2000 Levophed ischemic necrosis Mannitol – IV Methadone BJD 143:1356-1358, 2000 Methotrexate necrosis BMJ 299:980-981, 1989; S Afr Med J 72:888, 1987 Nafcillin – IV Norepinephrine – IV BJD 143:1356-1358, 2000

Oxymetazoline – intra-arterial injection AD 138:1296-1298, 2002 Parenteral nutrition formulation extravasation J Miss State Med Assoc 40:307-311, 1999 Phenergan injection Phenytoin – IV JAAD 28:360-363, 1993 Photodynamic therapy Photochem Photobiol 68:575-583, 1998 Potassium salts – IV Quinine sulfate – acral necrosis Hautarzt 51:332-335, 2000 Radio-opaque dye – IV Sertraline – giant bulla with necrosis BJD 150:164-166, 2004 Sodium bicarbonate – IV Sodium tetradecyl sulfate extravasation J Derm Surg Oncol 19:641-646, 1993 Streptokinase – IV BMJ 309:378, 1994 Toxic epidermal necrolysis JAAD 40:458-461, 1999; JAAD 23:870-875, 1990 Tumor necrosis factor J Inflamm 47:180-189, 1995 Vaccination – DPT – livedoid skin necrosis (Nicolau syndrome) BJD 137:1030-1031, 1997 Vasoconstricting agents Ghatan p.31, 2002, Second Edition Vasopressin BJD 143:1356-1358, 2000; Dermatology 195:271-273, 1997; Cutis 57:330-332, 1996; JAAD 15:393-398, 1986; scrotal and abdominal skin necrosis Dig Dis Sci 30:46-464, 1985 Vinorelbine extravasation Tumori 86:289-292, 2000

Acids and alkali Rook p.867, 1998, Sixth Edition Alcohol – percutaneous absorption and necrosis in a preterm infant Arch Dis Child 57:477-479, 1982 Antiseptics (isopropyl alcohol) – in newborn Pediatrics 68:587-588, 1981 Calcium – percutaneous calcium salts (calcium chloridecontaining EEG paste on abraded scalp skin) Ped Derm 15:27-30, 1998; Dermatologica 181:324-326, 1990 Collagen implant (bovine) – Zyderm or Zyplast JAAD 25:319-326, 1991; sterile abscesses with local necrosis JAAD 25:319-326, 1991 Dequalinium (quaternary ammonium antibacterial agent) – necrotizing ulcers of the penis Trans St John’s Hosp Dermatol Soc 51:46-48, 1965 Drug abuse NEJM 277:473-475, 1967 Orthopedic braces Am J Orthop 27:371-372, 1998 Hepatitis B virus vaccine – Arthus reaction Clin Inf Dis 33:906-908, 2001 Hydrofluoric acid Ann DV 122:512-513, 1995 Intra-arterial injections Rook p.2231, 1998, Sixth Edition Irritant contact dermatitis Naphtha (charcoal lighter fluid), subcutaneous injection Am J Emerg Med 16:508-511, 1998 Phosphoric acid Rook p.867, 1998, Sixth Edition PPD NEJM 295:1263, 1976 Sclerotherapy – high-concentration sclerotherapy for varicose veins Dermatol Surg 26:535-542, 2000 Silicone, injected AD 141:13-15, 2005; Derm Surg 27:198-200, 2001 Tibial cement extrusion J Arthroplasty 13:826-829, 1998 Tongue ring – mucosal necrosis

Acanthamoeba J Clin Inf Dis 20:1207-1216, 1995; perianal gangrene Trop Doct 12:162-163, 1982; Proc R Soc Med 66:677-678, 1973 Acremonium – target-like lesions with central necrosis Rook p.1375, 1998, Sixth Edition Actinomycosis Ghatan p.31, 2002, Second Edition Aeromonas hydrophilia sepsis African tick bite fever (Rickettsia africae) – crusted eschar BJD 143:1109-1110, 2000; hemorrhagic pustule, purpuric papules; transmitted by Amblyomma ticks) – high fever, arthralgia, myalgia, fatigue, rash in 2-3 days, with eschar, maculopapules, vesicles, and pustules JAAD 48:S18-19, 2003 Alternariosis Amebiasis Ghatan p.31, 2002, Second Edition Anthrax – eschar of the fingers, face, or neck Cutis 69:23-24, 2002; Cutis 67:488-492, 2001; Clin Inf Dis 19:1009-1014, 1994; Cutis 48:113-114, 1991; Cutis 40:117-118, 1987; eschar and ulceration NEJM 352:700-707, 2005; JAAD 47:766-769, 2002 Arthropod bite NEJM 352:700-707, 2005; hypersensitivity to mosquito bites with intense erythema, edema and necrosis AD 139:1601-1607 Aspergillosis NEJM 352:700-707, 2005; AD 141:633-638, 2005; Clin Inf Dis 22:1102-1104, 1996; A. flavus, primary cutaneous – necrotic ulcer AD 141:1035-1040, 2005; necrotizing dermal plaque Textbook of Neonatal Dermatology, p.229, 2001; BJD 85 (Suppl 17):95-97, 1971; primary cutaneous aspergillosis JAAD 38:797-798, 1998; Infect Control Hosp Epidemiol 17:365-366, 1996; primary cutaneous aspergillosis in premature infants Ped Derm 19:439-444, 2002; Aspergillus flavus JAAD 46:945-947, 2002; zosteriform A. flavus – black eschar JAAD 38:488-490, 1998; eschar and ulceration JAAD 53:213-219, 2005; JAAD 47:766-769, 2002 Bacteroides – non-clostridial gas gangrene in diabetics JAMA 233:958-963, 1975 BCG lymphadenopathy with eschar Ped Derm 21:646-651, 2004 Beetle bite J Cutan Med Surg 4:219-222, 2000 Bejel Ghatan p.31, 2002, Second Edition Bilophila wadsworthia and Escherichia coli – necrotizing fasciitis Boutonneuse fever Brown recluse spider bite NEJM 352:700-707, 2005 Brucellosis – necrotizing vasculitis Burkholderia pseudomallei (melioidosis) – disseminated; ecthyma-like lesions Clin Inf Dis 40:988-989, 1053-1054, 2005 Calymmatobacterium granulomatis (Donovanosis) – penile necrosis J Clin Inf Dis 25:24-32, 1997 Cancrum oris (noma) – labial and buccal necrosis J Dent Child 48:138-141, 1981 Candidiasis, disseminated – necrotic eschar and pustules; ecthyma gangrenosum-like lesion Rook p.2752, 1998, Sixth Edition Capnocytophaga canimorsus sepsis – dog and cat bites; necrosis with eschar Cutis 60:95-97, 1997; JAAD 33:1019-1029, 1995 Carbuncle Rook p.1119, 1998, Sixth Edition Caterpillars, poisonous Ann DV 125:489-491, 1998 Cellulitis, erysipelas Rook p.113, 1998, Sixth Edition Cheyletiella mites – dogs, cats; papulovesicles, pustules, necrosis JAAD 50:819-842, 2004; AD 116:435-437, 1980 Chromobacterium violaceum NEJM 352:700-707, 2005

Clostridium botulinum – wound botulism in drug addicts Clin Inf Dis 31:1018-1024, 2000 Clostridial cellulitis of the newborn AD 113:683-684, 1977 Clostridium perfringens (gas gangrene) – most common cause of necrotizing anorectal and perianal infection Surgery 86:655-662, 1979 Clostridium septicum – gas gangrene and myonecrosis Clostridium welchii Rook p.2188, 1998, Sixth Edition Coccidioidomycosis – primary cutaneous coccidioidomycosis; necrotic papule JAAD 49:944-949, 2003 Corynebacterium urealyticum Clin Inf Dis 22:853-855, 1996 Cowpox (feline orthopoxvirus) – eschar JAAD 49:513-518, 2003; BJD 145:146-150, 2001; Tyring p.52, 2002 Cryptococcosis NEJM 352:700-707, 2005 Diphtheria Ecthyma – eschar and ulceration JAAD 47:766-769, 2002 Ecthyma gangrenosum – Pseudomonas aeruginosa NEJM 352:700-707, 2005; JAAD 11:781-787, 1984; Pseudomonas cepacia AD 113:199-202, 1977; gram-negative bacteria Postgrad Med 106:249-250, 1999; AD 121:873-876, 1985; JAAD 11:781-787, 1984; Escherichia coli J Clin Gastroenterol 4:145-148, 1982; Rev Inf Dis 2:854-865, 1980; Aeromonas hydrophila NY State J Med 82:1461-1464, 1982; J Pediatr 83:100-101, 1973; Klebsiella pneumoniae Int J Dermatol 34:216-217, 1995; South Med J 84:790-793, 1991; Xanthomonas maltophiia Ann Intern Med 121:969-973, 1994; Morganella morganii Mil Med 153:400-401, 1988; Serratia marcescens Rev Inf Dis 2:854-865, 1980; Citrobacter freundii JAAD 50:S114-117, 2004; Aspergillus fumigatus; Aspergillus niger Am J Clin Pathol 72:230-232, 1979; Candida albicans Am J Med 70:1133-1135, 1982; Exserohilum species Ped Derm 20:495-497, 2003; Vibrio, Rhizopus, Fusarium; Scytalidium dimidiatum J Clin Microbiol Infect Dis 12:118-121, 1993; Pseudallescheria boydii, Curvularia sp Bone Marrow Transplant 27:1311-1313, 2001; herpes simplex Clin Infect Dis 29:454-455, 1999 Endocarditis, bacterial – necrosis in children AD 133:1500-1501, 1997 Epidemic typhus (Rickettsia prowazeki) – pink macules on sides of trunk, spreads centrifugally; flushed face with injected conjunctivae; then rash becomes deeper red, then purpuric; gangrene of finger, toes, genitalia, nose JAAD 2:359-373, 1980 Erysipelothrix rhusiopathiae – rare systemic form with localized swellings with central necrosis Rook p.1138, 1998, Sixth Edition Escherichia coli – non-clostridial gas gangrene in diabetics JAMA 233:958-963, 1975 Flavobacterium odoratum – necrotizing fasciitis J Clin Inf Dis 21:1337-1338, 1995 Fournier’s gangrene Rook p.3176, 1998, Sixth Edition Fusarium – sepsis; red-gray macules or papules with central eschar JAAD 47:659-666, 2002; Ped Derm 9:62-65, 1992; Fusarium solanae – target-like lesions with central necrosis; violaceous nodules with central necrosis Clin Inf Dis 32:1237-1240, 2001; Rook p.1375, 1998, Sixth Edition; Eur J Clin Microbiol Infect Dis 13:152-161, 1994 Fusobacterium – abscesses with necrosis Rook p.1157, 1998, Sixth Edition Gangrenous and crepitant cellulitis

Clostridium perfringens Infected vascular gangrene Non-clostridial crepitant cellulitis (anaerobes) Phycomycotic gangrenous cellulitis Progressive bacterial synergistic gangrene Vibrio vulnificus

Gemella morbillorum – necrotizing fasciitis JAAD 52:704-705, 2005 Glanders – eschar and ulceration JAAD 47:766-769, 2002 Gonococcemia NEJM 352:700-707, 2005; periarticular lesions appear in crops with red macules, papules, vesicles with red halo, pustules, bullae becoming hemorrhagic and necrotic; suppurative arthritis and tenosynovitis Ann Intern Med 102:229-243, 1985; NEJM 282:793-794, 1970; in children AD 133:1500-1501, 1997 Gypsy moth caterpillar reaction Hepatitis C – with cryoglobulins; thrombotic vasculitis AD 131:1185-1193, 1995 Herpes simplex – necrotizing balanitis JAMA 248:215-216, 1982; Br J Vener Dis 55:48-51, 1979; necrotic digits in chronic herpes simplex; neonatal herpes simplex – necrotic plaque of scalp at site of fetal monitor electrode placement Textbook of Neonatal Dermatology, p.204, 2001 Herpes zoster – cutaneous necrosis; jaw necrosis Oral Surg 56:39-46, 1983 Histoplasmosis – necrotic papules and ulcers BJD 113:345-348, 1985 HIV disease – polyarteritis-like vasculitis with acral necrosis J Clin Inf Dis 23:659-661, 1996 Insect bite – especially in Hodgkin’s disease; eschar and ulceration JAAD 47:766-769, 2002 Klebsiella sepsis – including non-clostridial gas gangrene in diabetics JAMA 233:958-963, 1975 Leishmaniasis – eschar and ulceration NEJM 352:700-707, 2005; JAAD 47:766-769, 2002; necrosis of lips Mycoses 41 Suppl 2:78-80, 1998 (German); JAAD 28:495-496, 1993; Dermatologica 150:292-294, 1975; Leishmania aethiopica – lip edema Trans R Soc Trop Med Hyg 63:708-737, 1969; espundia (mucocutaneous leishmaniasis) – nasopharyngeal mutilation with protuberant lips Am J Trop Med Hyg 59:49-52, 1998 Leprosy – plantar necrotic blister; Lucio’s phenomenon – bullae and necrosis leaving deep painful ulcers Int J Lepr 47:161-166, 1979; stellate necrosis in Lucio’s phenomenon JAAD 51:416-426, 2004; lepromatous leprosy including erythema nodosum leprosum (vasculitis) JAAD 51:416-426, 2004; AD 111:1575-1580, 1975; eschar and ulceration JAAD 47:766-769, 2002 Lyme disease – vesicular variant with secondary necrosis NEJM 352:700-707, 2005; JAAD 49:363-392, 2003 Malaria Mediterranean spotted fever AD 139:1545-1552, 2003 Melioidosis – ecthyma gangrenosum-like lesions AD 135:311-322, 1999 Meningococcemia Ann Plast Surg 46:199-200, 2001; purpura fulminans Burns 24:272-274, 1998; chronic meningoccemia Ped Derm 13:483-487, 1996 Milker’s nodule – eschar and ulceration JAAD 47:766-769, 2002 Mosquito bite hypersensitivity syndrome in EBV-associated natural killer cell leukemia/lymphoma – clear or hemorrhagic bullae with necrosis, ulceration and scar formation JAAD 45:569-578, 2001 Mucormycosis – Rhizopus oryzae, Absidia corymbifera, Cunninghamella berthelletiae, Rhizomucor pusillus BJD 150:1212-1213, 2004; Mucor circinelloides, Apophysomyces elegans, Saksanaea vasiformis; face, scalp, extremities – necrotic ulcer Ped Derm 20:411-415, 2003; AD 136:1165-1170, 2000; AD 133:249-251, 1997; J Clin Inf Dis 19:67-76, 1994; palatal ulcer Oral Surg 68:624-627, 1989; eschar and ulceration JAAD 47:766-769, 2002; rhino-orbital mucormycosis – palatal and nasal mucosal necrosis NEJM 341:265-273, 1999

Mycetoma JAAD 32:897-900, 1995 Mycobacterium chenonei var. abscessus Mycobacterium haemophilum – necrotic ulcer J Infection 23:303-306, 1991 Mycobacterium kansasii – papulonecrotic tuberculid JAAD 40:359-363, 1999 Mycobacterium marinum Tyring p.55, 2002 Mycobacterium tuberculosis NEJM 352:700-707, 2005; primary inoculation Tyring p.55, 2002; miliary tuberculosis – large crops of blue papules, vesicles, pustules, hemorrhagic papules; red nodules; vesicles become necrotic to form ulcers JAAD 50:S110-113, 2004; Practitioner 222:390-393, 1979; Am J Med 56:459-505, 1974; AD 99:64-69, 1969; lupus vulgaris; starts as red-brown plaque, enlarges with serpiginous margin or as discoid plaques; vegetating forms – ulcerate, areas of necrosis, invasion of mucous membranes with destruction of cartilage (lupus vorax); head, neck, around nose, extremities, trunk Int J Dermatol 26:578-581, 1987; Acta Tuberc Scand 39 (Suppl 49):1-137, 1960; lupus vulgaris, miliary, papulonecrotic tuberculid Dermatologica 173:189-195, 1986; BJD 91:263-270, 1974; congenital tuberculosis – red papule with central necrosis AD 117:460-464, 1981; penile gangrene Rook p.3194, 1998, Sixth Edition; eschar and ulceration JAAD 47:766-769, 2002 Mycobacterium ulcerans NEJM 352:700-707, 2005 Mycoplasma pneumoniae – purpura and necrosis Clin Exp Immunol 14:531-539, 1973 Necrolytic acral erythema – hepatitis C infection Int J Dermatol 35:252-256, 1996 Necrotizing fasciitis – Streptococcus pyogenes Curr Prob Dermatol 14:183-220, 2002; Ann DV 128:376-381, 2001; methicillin-resistant Staphylococcus aureus NEJM 352:1445-1453, 2005; Streptococcus pneumoniae – due to intramuscular injection Clin Inf Dis 33:740-744, 2001; Serratia marcescens Clin Inf Dis 23:648-649, 1996; JAAD 20:774-778, 1989; Bacteroides spp.in penile necrotizing fasciitis JAAD 37:1-24, 1997; neonatal Pediatrics 103:53, 1999; in infancy Ped Derm 2:55-63, 1984; Clostridial cellulitis (gangrene); progressive synergistic gangrene; gangrenous cellulitis (Pseudomonas); Fournier’s gangrene Rook p.1164, 1998, Sixth Edition; periorbital AD 140:664-666, 2004 Nocardia asteroides AD 121:898-900, 1985; J Inf Dis 134:286-289, 1976 Noma (cancrum oris) (necrotizing gingivitis) – Fusobacterium necrophorum, Prevotella intermedia, α-hemolytic streptococci, Actinomyces spp.Oral Dis 5:144-149,156-162, 1999 Noma neonatorum – Pseudomonas infection Ped Inf Dis 21:83-84, 2002 Non-clostridial gas gangrene – cellulitis with necrosis; Streptococcus angiosus, Streptococcus faecalis, aerobic streptococci, E. coli, Proteus, Bacteroides, Klebsiella spp. JAAD 40:347-349, 1999; Diabetalogia 13:373-376, 1977 North American blastomycosis Ghatan p.31, 2002, Second Edition North Asian tick typhus Orf – eschar and ulceration JAAD 47:766-769, 2002; Tyring p.53, 2002 Paecilomyces lilacinus – red nodules with necrotic centers Ann Intern Med 125:799-806, 1996; ecthyma gangrenosum-like JAAD 39:401-409, 1998 Penicillium marneffei – necrotic papules and/or nodules JAAD 37:450-472, 1997; Clin Inf Dis 23:125-130, 1996; Lancet 344:110-113, 1994; Mycoses 34:245-249, 1991 Phaeoacremonium inflatipes – fungemia in child with aplastic anemia; swelling and necrosis of lips, periorbital edema, neck swelling Clin Inf Dis 40:1067-1068, 2005

Phaeohyphomycosis, subcutaneous Ped Inf Dis 5:380-382, 1986; phaeomycotic cyst of the hand – Phialophora, Cladosporium, Alternaria JAAD 8:1-16, 1983 Phagedenic balanitis Rook p.3194, 1998, Sixth Edition Phlegmon – necrotic cutaneous phlegmon Plague (Yersinia pestis) – ecthyma gangrenosum-like lesions at site of initial flea bite; eschars AD 135:311-322, 1999; Clin Inf Dis 19:655-663, 1994; eschar and ulceration JAAD 47:766-769, 2002 Portuguese man-of-war stings J Emerg Med 10:71-77, 1992 Pseudallescheria boydii – mycetoma – bulla with central necrosis AD 132:382-384, 1996 Pseudomonas – ecthyma gangrenosum in Pseudomonas sepsis; eschar and ulceration JAAD 47:766-769, 2002; Textbook of Neonatal Dermatology, p.193, 2001; JAAD 11:781-787, 1984; Arch Int Med 128:591-595, 1971; noma neonatorum – gangrenous changes in oronasal and perineal areas, scrotum, and eyelids (Pseudomonas in infants) Lancet 2:289-291, 1978; Pseudomonas sepsis – penile gangrene J Urol 124:431-432, 1980; non-clostridial gas gangrene in diabetics JAMA 233:958-963, 1975 Pythium insidiosum (pythiosis) (alga) – ascending gangrene of legs; Thailand J Infect Dis 159:274-280, 1989; cellulitis, infarcts, ulcers JAAD 52:1062-1068, 2005 Queensland tick typhus AD 139:1545-1552, 2003 Rat bite fever (Sodoku) – Spirillium minor – eschar (acrally) Ann Emerg Med 14:1116-1118, 1985; eschar and ulceration JAAD 47:766-769, 2002 Rhizopus AD 125:952-956, 1989; Rhizopus azygosporus BJD 153:428-430, 2005 Ricketsial pox (Rickettsia akari ) (Kew Gardens spotted fever) – house mouse mite bite (Liponyssus (Allodermanyssus) sanguineus) NY Med 2:27-28, 1946; eschars in rickettsial pox, R. conorii, R. sibirica, R. australis, R. japonicum AD 139:1545-1552, 2003; Clin Inf Dis 18:624-626, 1994; eschar and ulceration JAAD 47:766-769, 2002 Rocky Mountain spotted fever JAAD 49:363-392, 2003; Clin Inf Dis 16:629-634, 1993; eschar at bite site; DIC J Clin Inf Dis 21:429, 1995; massive skin necrosis South Med J 71:1337-1340, 1978 Scopulariopsis Scorpion sting Scrub typhus (Rickettsia tsutsugamuchi) (mites) – headache and conjunctivitis; eschar with black crust; generalized macular or morbilliform rash AD 139:1545-1552, 2003; JAAD 2:359-373, 1980; eschar and ulceration JAAD 47:766-769, 2002 Sea anemone sting Serratia marcescens – including necrotizing fasciitis J Clin Inf Dis 23:648-649, 1996 Siberian tick typhus AD 139:1545-1552, 2003 Smallpox vaccination Clin Inf Dis 37:241-250, 2003; progressive vaccinia Clin Inf Dis 37:251-271, 2003 Snake bites – edema, erythema, pain, and necrosis NEJM 347:347-356, 2002; green pit viper Am J Trop Med 58:22-25, 1998; spitting cobra (Naja nigricollis) Toxicon 25:665-672, 1987 Spider bites Trans R Soc Trop Med Hyg 92:546-548, 1998; South Med J 69:887-891, 1976; brown recluse spider bite JAAD 44:561-573, 2001; eschar and ulceration JAAD 47:766-769, 2002 Sporotrichosis NEJM 352:700-707, 2005; Tyring p.55, 2002; Ghatan p.31, 2002, Second Edition Staphylococcus aureus NEJM 352:700-707, 2005; Staphylococcus aureus purpura fulminans and toxic shock syndrome Clin Inf Dis 40:941-947, 2005

Staphylococcal scalded skin syndrome Stingray bite BJD 143:1074-1077, 2000 Streptococcus – acute necrotizing infection following bites or scratches of dogs or cats NEJM 352:700-707, 2005; Ann DV 123:804-806, 1996 Streptococcus pneumoniae Clin Inf Dis 21:697-698, 1995; penile gangrene Rook p.3194, 1998, Sixth Edition Subacute bacterial endocarditis – Streptococcus viridans with cutaneous vasculitis Synergistic necrotizing gangrene (Meleney’s synergistic gangrene) Rook p.2266, 1998, Sixth Edition; Surgery 86:655-662, 1979; Arch Surg 9:317-364, 1924 Syphilis – malignant lues NEJM 352:700-707, 2005; JAAD 22:1061-1067, 1990; penile gangrene Rook p.3194, 1998, Sixth Edition Tanapox – umbilicated papule progressing to necrosis JAAD 44:1-14, 2001 Tick bites – especially soft ticks JAAD 49:363-392, 2003 Tick typhus (Boutonneuse fever, Kenya tick typhus, African and Indian tick typhus) (ixodid ticks) – small ulcer at site of tick bite (tache noire) – black necrotic center with red halo; pink morbilliform eruption of forearms, then generalizes, involving face, palms, and soles; may be hemorrhagic; recovery uneventful JAAD 2:359-373, 1980; eschar and ulceration JAAD 47:766-769, 2002 Trichosporon beigelii AD 129:1020-1023, 1993 Tropical ulcer – eschar and ulceration JAAD 47:766-769, 2002 Trypanosoma brucei rhodiense – necrotic chancre J Clin Inf Dis 23:847-848, 1996 Tsutsugamushi fever Dtsch Med Wochenschr 123:562-566, 1998 Tularemia – necrotic papule Cutis 54:279-286, 1994; eschar and ulceration NEJM 352:700-707, 2005; JAAD 47:766-769, 2002; skin slough from hamster bite MMWR 53:1202-1203, 2005 Vaccinia – progressive vaccinia J Clin Inf Dis 25:911-914, 1997 Varicella – varicella gangrenosa Tyring p.124, 2002; Arch Dis Child 30:177-179, 1955; chronic varicella zoster virus in AIDS AD 124:1011-1012, 1988; penile gangrene in infancy Rook p.3194, 1998, Sixth Edition; atypical recurrent varicella with vesiculopapular lesions with central necrosis JAAD 48:448-452, 2003 Venoms Vibrio vulnificus sepsis JAAD 46:S144-145, 2002; Ann DV 128:653-655, 2001; JAAD 24:397-403, 1991 Xanthomonas maltophilia – ecthyma gangrenosum-like lesions JAAD 37:836-838, 1997 Yaws Zygomycosis AD 140:877-882, 2004; JAAD 32:346-351, 1995; Rhizopus arrhizus; bull’s eye infarct JAAD 51:996-1001, 2004; JAMA 2254:737-738, 1973; Apophysomyces elegans, Saksenaza vasiformis J Clin Inf Dis 24:580-583, 1997; J Clin Inf Dis 19:67-76, 1994

Langerhans cell histiocytosis NEJM 352:700-707, 2005

Dermatitis gangrenosum infantum – multiple necrotic ulcers complicating varicella, seborrheic dermatitis BJD 75:206-211, 1963 Erythema multiforme Medicine 68:133-140, 1989; JAAD 8:763-765, 1983; Stevens-Johnson syndrome Rook p.3365-3366, 1998, Sixth Edition

Nodular panniculitis, idiopathic – overlying necrosis with drainage of oily brown serous fluid Rook p.2410-2411, 1998, Sixth Edition; Medicine 64:181-191, 1985 Pyoderma gangrenosum NEJM 352:700-707, 2005; Br J Plast Surg 53:441-443, 2000; JAAD 18:559-568, 1988 Pyoderma gangrenosum-like lesions, polyarthritis, and lung cysts with ANCA to azurocidin – umbilicated necrotic lesions Clin Exp Immunol 103:397-402, 1996 Sarcoid – resembling papulonecrotic tuberculid AD Syphilol 13:675-676, 1926

Acrodermatitis enteropathica – black necrotic lesions Rook p.2670, 1998, Sixth Edition Calcific uremic arteriolopathy NEJM 352:700-707, 2005 Calcinosis cutis – metastatic calcification AD 106:398-402, 1972 Calciphylaxis (vascular calcification cutaneous necrosis syndrome) AD 140:1045-1048, 2004; J Dermatol 28:27-31, 2001; Br J Plast Surg 53:253-255, 2000; J Cutan Med Surg 2:245-248, 1998; JAAD 40:979-987, 1999; JAAD 33:53-58, 1995; JAAD 33:954-962, 1995; JAAD 33:954-962, 1995; AD 127:225-230, 1991 Cold agglutinins BJD 139:1068-1072, 1998; JAAD 19:356-357, 1988; at site of transfusion JAAD 19:356-357, 1988; intravascular coagulation necrosis JAAD 25:882-888, 1991 Cryofibrinogenemia Am J Med 116:332-337, 2004; Clin Exp Dermatol 25:621-623, 2000; Am J Kidney Dis 32:494-498, 1998; AD 133:1500-1501, 1997; JAAD 24:342-345, 1991; intravascular coagulation necrosis JAAD 25:882-888, 1991 Cryoglobulins – intravascular coagulation necrosis NEJM 352:700-707, 2005; JAAD 25:882-888, 1991; necrotic ears BJD 143:1330-1331, 2000; monoclonal cryoglobulinemia; cutaneous necrosis JAAD 48:311-340, 2003 Diabetes – microangiopathy, neuropathy NEJM 352:700-707, 2005; dry or wet gangrene; arteriosclerotic peripheral vascular disease Caputo p.189, 2000; Rook p.2231,2674, 1998, Sixth Edition; penile gangrene J Urol 132:560-562, 1984 Homocystinuria – cystathionine-beta synthase deficiency; distal cutaneous necrosis Ann DV 126:822-825, 1999 Hyperparathyroidism Hyperphosphatemia J Parent Enteral Nutr 21:50-52, 1997 Hyperviscosity – cryos, paraproteinemia, dehydration Oxalosis – acral necrosis with livedo; (primary oxalosis (hyperoxaluria) – type 1 – alanine glyoxalate aminotransferase (transaminase) deficiency; chromosome 2q36-37; type 2 (rare) – D-glyceric acid dehydrogenase deficiency AD 137:957-962, 2001; JAAD 22:952-956, 1990; AD 131:821-823, 1995; primary hyperoxaluria; necrosis with limb gangrene JAAD 49:725-728, 2003; livedo reticularis, ulcers, and peripheral gangrene AD 136:1272-1274, 2000 Paroxysmal nocturnal hemoglobinuria – petechiae, ecchymoses, red plaques which become hemorrhagic bullae with necrosis; lesions occur on legs, abdomen, chest, nose, and ears; deficiency of enzymes – decay-accelerating factor (DAF) and membrane inhibitor of reactive lysis (MIRL) AD 138:831-836, 2002; AD 122:1325-1330, 1986; AD 114:560-563, 1978 Protein C deficiency Blood Coagul Fibrinolysis 9:351-354, 1998; AD 133:1500-1501, 1997; Semin Thromb Hemost 16:299-309, 1990; Blood Coagul Fibrinolysis 1:319-330, 1990; AD 123:1701a-1706a, 1987

Protein S deficiency JAAD 29:853-857, 1993; Semin Thromb Hemost 16:299-309, 1990 Prothrombin G20210A – mutation (heterozygous) Pediatr Hematol Oncol 16:561-564, 1999 Purpura fulminans, neonatal – purpura or cellulitis-like areas evolving into necrotic bullae or ulcers Textbook of Neonatal Dermatology, p.151, 2001 Short bowel syndrome – superficial skin necrosis Harefuah 136:855-857,915, 1999 Sickle-cell anemia – stellate necrosis Thrombocythemia – livedo reticularis, acrocyanosis, erythromelalgia, gangrene, pyoderma gangrenosum Leuk Lymphoma 22 Suppl 1:47-56, 1996; Br J Haematol 36:553-564, 1977; AD 87:302-305, 1963; essential thrombocythemia with or without necrotizing vasculitis JAAD 24:59-63, 1991

Atrial myxoma Am J Med 62:792-794, 1977 Basal cell carcinoma NEJM 352:700-707, 2005 Eosinophilic histiocytosis JAAD 13:952-958, 1985 Fibrosarcoma/spindle cell sarcoma – necrotic red or violaceous nodule Rook p.2352, 1998, Sixth Edition Histiocytic lymphoma (true histiocytic lymphoma) JAAD 50:S9-10, 2004 Kaposi’s sarcoma Keloids – suppurative necrosis Rook p.2056-2057, 1998, Sixth Edition Leukemia cutis NEJM 352:700-707, 2005; BJD 143:773-779, 2000 Lymphoma – cutaneous T-cell lymphoma NEJM 352:700-707, 2005; JAAD 47:914-918, 2002; hydroa vacciniforme-like primary CD8 + angiocentric lymphoma associated with Epstein-Barr virus BJD 147:587-591, 2002; JAAD 38:574-579, 1998; AD 133:1156-1157, 1997; subcutaneous panniculitis-like T-cell lymphoma with necrotic nodules of legs AD 141:1035-1040, 2005; Am J Surg Pathol 15:17-27, 1991; primary cutaneous CD30 + lymphoproliferative disorder (CD8 +/CD4 +) – necrotic nodule JAAD 51:304-308, 2004 Lymphomatoid papulosis – papules or nodules with central necrosis NEJM 352:700-707, 2005; JAAD 49:1049-1058, 2003; Am J Dermatopathol 18:221-235, 1996; JAAD 17:632-636, 1987; JAAD 13:736-743, 1985 Melanoma NEJM 352:700-707, 2005 Polycythemia vera Rook p.2231, 1998, Sixth Edition Squamous cell carcinoma NEJM 352:700-707, 2005

Hypersensitivity to mosquito bites – associated with lymphoma/leukemia BJD 153:210-212, 2005; JAAD 45:569-578, 2001; BJD 138:905-906, 1998 Paraneoplastic acral vascular syndrome – acral cyanosis and gangrene JAAD 47:47-52, 2002; AD 138:1296-1298, 2002 Paraneoplastic pemphigus – necrosis of eyelids and oral mucosa Great Cases from the South, AAD Meeting, March 2000 Pyoderma gangrenosum, atypical (bullous) – associated with myeloproliferative disease Venous thrombosis

Hydroa vacciniforme BJD 144:874-877, 2001; AD 118:588-591, 1982

Acne necrotica varioliformis Acute parapsoriasis (pityriasis lichenoides et varioliformis acuta) (Mucha-Habermann disease) JAAD 51:606-624, 2004; AD 123:1335-1339, 1987; AD 118:478, 1982; febrile ulceronecrotic variant of Mucha-Habermann disease Ped Derm 22:360-365, 2005; BJD 152:794-799, 2005; JAAD 49:1142-1148, 2003; BJD 147:1249-1253, 2002; Ped Derm 8:51-57, 1991; Ann DV 93:481-496, 1966 Erythema elevatum diutinum BJD 67:121-145, 1955 Pityriasis rosea – vesicular variant Pustular psoriasis Pyoderma faciale Subcutaneous fat necrosis of newborn

Factitial dermatitis – linear lesions Ped Derm 21:205-211, 2004; Rook p.2800-2802, 1998, Sixth Edition; JAAD 1:391-407, 1979; penile gangrene Rook p.3194, 1998, Sixth Edition

Anterior tibial syndrome Antiphospholipid antibody syndrome – eschar and ulceration NEJM 352:700-707, 2005; JAAD 47:766-769, 2002; NEJM 346:752-763, 2002; Semin Arthritis Rheum 31:127-132, 2001; JAAD 36:149-168, 1997; JAAD 36:970-982, 1997; Clin Rheumatol 15:394-398, 1996; South Med J 88:786-788, 1995; BJD 120:419-429, 1989; nasal tip necrosis BJD 142:1199-1203, 2000; lupus anticoagulant Behçet’s disease (bullous necrotizing vasculitis) JAAD 21:327-330, 1989; pyoderma gangrenosum-like lesions JAAD 40:1-18, 1999 Hypereosinophilic syndrome – cutaneous necrotizing eosinophilic vasculitis with Raynaud’s phenomenon BJD 143:641-644, 2000; hypereosinophilic syndrome associated with T-cell lymphoma JAAD 46:S133-136, 2002; fingertip necrosis with vasculitis AD 132:535-541, 1996; cutaneous infarction BJD 148:817-820, 2003 Infantile myofibromatosis Ped Derm 5:37-46, 1988 Neurofibromatosis type I – vasculopathy with acral necrosis Pediatrics 100:395-397, 1997 POEMS syndrome – vasculitis with necrosis Racand syndrome – acral or digital necrosis; Raynaud’s phenomenon, anticentromere antibodies and necrosis of digits without sclerodactyly and sclerosis of internal organs JAAD 43:621, 2001 Rowell’s syndrome – lupus erythematosus and erythema multiforme-like syndrome – papules, annular targetoid lesions, vesicles, bullae, necrosis, ulceration, oral ulcers; perniotic lesions JAAD 21:374-377, 1989 Sjögren’s syndrome – nail-fold infarcts, gangrene of fingers Rook p.2572, 1998, Sixth Edition Thoracic outlet obstruction AD 138:1296-1298, 2002 Werner’s syndrome – acral necrosis Medicine 45:177-221, 1966 Wiskott-Aldrich syndrome

Carbon monoxide – endothelial cell damage

Blunt trauma – repetitive blunt trauma AD 138:1296-1298, 2002 Chemical burns

Acids and alkalis Cement (calcium hydroxide) BJD 102:487-489, 1980; Br Med J i:1250, 1978 Chromic acid Rook p.868, 1998, Sixth Edition Hydrofluoric acid Lime dust – necrosis with ulcers Contact Dermatitis 1:59, 1981 Phenol Phosphorus

Chilblains – fingertips and toetips; with necrosis on fingers, toes, nose, and ears in patients with monocytic leukemia AD 121:1048, 1052, 1985 Coma bullae – sweat gland necrosis Ann DV 122:780-782, 1995 Compartmental syndrome (crush injury of thorax) – skin necrosis Condom catheter – gangrene of glans or shaft of penis JAMA 244:1238, 1980 Crush injury of fingertip AD 138:1296-1298, 2002 Cryosurgery JAAD 8:513-519, 1983 Electric shock – fingertip necrosis AD 138:1296-1298, 2002 External compression of arterial supply (popliteal entrapment, cervical rib) Rook p.2231, 1998, Sixth Edition Frostbite – vesicles, bullae, ischemic necrosis; calcification Rook p.3021, 1998, Sixth Edition Gentian violet Acta DV 52:55-60, 1972 Heel sticks of neonate – gangrene of heel Eichenfeld p.105, 2001 Hypothenar hammer syndrome AD 138:1296-1298, 2002 Intravenous drug addiction – dorsal vein of penis; necrosis BJD 150:1-10, 2004; Cutis 29:62-72, 1981 Laser burns Rook p.953, 1998, Sixth Edition Microwave radiation burns Rook p.953, 1998, Sixth Edition Nerve injury, traumatic – small areas of acral necrosis Rook p.2776, 1998, Sixth Edition Negative pressure device for erectile impotence J Urol 146:1618-1619, 1991 Orthopedic braces Injury 25:323-324, 1994 Oxygen face mask with continuous positive pressure Anaesthesia 48:147-148, 1993 Physical trauma Post-surgical – penile gangrene Rook p.3194, 1998, Sixth Edition Pressure necrosis NEJM 352:700-707, 2005 Pulse oximetry – acral necrosis Cutis 48:235-237, 1991; Anesth Analg 67:712-713, 1988 Radial or ulnar artery cannulation AD 138:1296-1298, 2002; N Y State J Med 90:375-376, 1990 Radiation – necrosis NEJM 352:700-707, 2005; J Laryngol Otol 112:1142-1146, 1998; necrosis after fluoroscopy during coronary angioplasty AD 139:140-142, 2003 Skin popping Clin Nucl Med 22:865-866, 1997 Surgery – pressure necrosis of scalp, hips Surgical embolization – occipital scalp necrosis and scarring Surg Neurol 25:357-366, 1988

Tourniquet injury (hair) – penile gangrene Rook p.3194, 1998, Sixth Edition Traumatic disruption of arterial wall Rook p.2231, 1998, Sixth Edition Trench foot, immersion foot – superficial gangrene Rook p.959, 1998, Sixth Edition

Acute hemorrhagic edema of infancy – purpura in cockade pattern of face, cheeks, eyelids, and ears; may form reticulate pattern; edema of penis and scrotum JAAD 23:347-350, 1990; necrotic lesions of the ears, urticarial lesions; oral petechiae JAAD 23:347-350, 1990; Ann Pediatr 22:599-606, 1975; edema of limbs and face Cutis 68:127-129, 2001; AD 133:1500-1501, 1997 ANCA-associated vasculitis with acquired protein S deficiency Throm Haemost 84:929-930, 2000 Aneurysm – dissecting aneurysm, thrombosed aneurysm Rook p.2231, 1998, Sixth Edition Angiopericytomatosis (angiomatosis with cryoproteins) – painful red papules and ulcerated plaques acrally; necrotic plaques JAAD 49:887-896, 2003 Arterial fibromuscular dysplasia – fingertip necrosis AD 138:1296-1298, 2002 Arteriovenous fistulae – fingertip necrosis; vascular steal syndrome in hemodialysis patients with arteriovenous fistulae AD 138:1296-1298, 2002; Rook p.2731, 1998, Sixth Edition Atherosclerosis – ischemic necrosis, gangrene; leg ulcers with small areas of necrosis along margin AD 138:1296-1298, 2002; Rook p.2231,2265, 1998, Sixth Edition Atrophie blanche AD 119:963-969, 1983 Benign (reactive) angioendotheliomatosis – red-brown or violaceous nodules or plaques with small areas of necrosis JAAD 38:143-175, 1998 Churg-Strauss disease AD 139:715-718, 2003; JAAD 47:209-216, 2002; necrotic purpura of scalp Ann DV 122:94-96, 1995; umbilicated nodules with central necrosis of elbows and knees (papulonecrotic lesions) JAAD 48:311-340, 2003; BJD 127:199-204, 1992; skin infarcts BJD 150:598-600, 2004; Mayo Clinic Proc 52:477-484, 1977 Embolism, septic NEJM 352:700-707, 2005 Degos’ disease (malignant atrophic papulosis) BJD 100:21-36, 1979; Ann DV 79:410-417, 1954 Disseminated intravascular coagulation (DIC) – intravascular coagulation necrosis JAAD 25:882-888, 1991; peripheral symmetric gangrene AD 137:139-140, 2001 Emboli – tumor; intravascular coagulation necrosis; cholesterol emboli BJD 146:511-517, 2002; Medicine 74:350-358, 1995; JAAD 25:882-888, 1991; Angiology 38:769-784, 1987; AD 122:1194-1198, 1986; Angiology 37:471-476, 1986 Erythromelalgia – associated with thrombocythemia – may affect one finger or toe; ischemic necrosis JAAD 22:107-111, 1990 Giant cell arteritis – lip necrosis J Oral Maxillofac Surg 51:581-583, 1993; tongue necrosis Rook p.3120, 1998, Sixth Edition Glomeruloid angioendotheliomatosis – red purpuric patches and acral necrosis – associated with cold agglutinins JAAD 49:887-896, 2003 Hemangioma (proliferating hemangioma) – focal necrosis Henoch-Schönlein purpura Ped Derm 15:357-359, 1998; Ped Derm 12:314-317, 1995; Am J Dis Child 99:833-854, 1960; in the adult AD 125:53-56, 1989

Heterozygous factor V Leiden deficiency BJD 143:1302-1305, 2000 Hypertensive ulcer (Martorell’s ulcer) – starts as area of cyanosis with progression to ulcer of lower lateral leg with livedo at edges Phlebology 3:139-142, 1988 Hypotension Intravascular thrombosis – DIC (meningococcemia, Rocky Mountain spotted fever, fungal, parasitic (Strongyloides)), atheroemboli, fat emboli, calciphylaxis, coumarin necrosis, protein C or S deficiency, antithrombin III deficiency, purpura fulminans, antiphospholipid antibody syndrome, thrombotic vasculitis (LE), cryoglobulins, cryofibrinogens, oxalosis Ischemic venous thrombosis (phlegmasia cerulia dolens) (venous gangrene) JAAD 28:831-835, 1993; AD 123:933-936, 1987 Juvenile gangrenous vasculitis of the scrotum Rook p.3199, 1998, Sixth Edition Kasabach-Merritt syndrome Livedoid vasculopathy NEJM 352:700-707, 2005 Neonatal gangrene – secondary to umbilical arterial catheterizations, polycythemia, prematurity BJD 150:357-363, 2004 Perinatal gangrene of the buttock, scrotum, and prepuce AD 121:23-24, 1985 Polyarteritis nodosa – livedoid necrosis NEJM 352:700-707, 2005; peripheral embolization of thrombi with necrosis of fingers or toes Rook p.2212, 1998, Sixth Edition; cutaneous – peripheral gangrene Ped Derm 15:103-107, 1998; Ann Rheum Dis 54:134-136, 1995; JAAD 31:54-56, 1994 Purpura fulminans Semin Thromb Hemost 16:333-340, 1990; neonatal AD 123:1701a-1706a, 1987; neonatal purpura fulminans – ecchymoses of limbs at sites of pressure in first day of life; enlarge rapidly, hemorrhagic bullae with central necrosis; homozygous protein C or protein S deficiency Semin Thromb Hemost 16:299-309, 1990 Pustular vasculitis – annular pustular plaques with central necrosis Rook p.2167, 1998, Sixth Edition Radial artery removal for coronary bypass grafting AD 138:1296-1298, 2002 Raynaud’s phenomenon Reactive angioendotheliomatosis – red purple-purpuric patches and plaques with necrotic ulcers; includes acroangiomatosis, diffuse dermal angiomatosis, intravascular histiocytosis, glomeruloid angioendotheliomatosis, angiopericytomatosis (angiomatosis with luminal cryoprotein deposition), reactive angiomatosis-like reactive angioendotheliomatosis; associated with subacute bacterial endocarditis, hepatitis, cholesterol emboli, cryoglobulinemia, arteriovenous shunt, antiphospholipid antibody syndrome, chronic lymphocytic leukemia, monoclonal gammopathy, chronic renal failure, rheumatoid arthritis, severe peripheral vascular disease, arteriovenous fistulae JAAD 49:887-896, 2003; BJD 147:137-140, 2002; JAAD 42:903-906, 2000 Recurrent cutaneous eosinophilic necrotizing vasculitis – papules, purpura, necrosis Acta DV 80:394-395, 2000 Sickle-cell infarct – thrombotic vasculitis Small vessel occlusive arterial disease – diabetes NEJM 352:700-707, 2005 Subacute bacterial endocarditis with vasculitis Symmetric peripheral gangrene Cutis 46:53-55, 1990

Primary arterial disease – polyarteritis nodosa, SLE, rheumatoid arthritis, arteriosclerosis obliterans, thromboangiitis obliterans

Congestive heart failure Overwhelming infection – meningococcemia, pneumococcal, rickettsial, viral, fungal, other Miscellaneous – carbon monoxide poisoning, fibrin thrombi, cold injury, crutch pressure arteritis Mitral stenosis with or without left atrial wall thrombus Embolization – cholesterol, infectious, tumor, thromboembolic Myocardial infarction Pulmonary embolus Vasospastic – ergotism, Raynaud’s Primary venous disease – venous gangrene

Syphilitic aortic aneurysm eroding through the sternum Dur M Cardiothorac Surg 10:922-924, 1996 Takayasu’s arteritis – cutaneous necrotizing vasculitis NEJM 352:700-707, 2005; Dermatology 200:139-143, 2000 Temporal arteritis (giant cell arteritis) Arthritis Rheum 42:1296, 1999; with scalp necrosis BJD 120:843-846, 1989; gangrene of leg, tongue necrosis BJD 151:721-722, 2004; BJD 76:299-308, 1964 Thromboangiitis obliterans (Buerger’s disease) Rook p.2233, 1998, Sixth Edition; Am J Med Sci 136:567-580, 1908 Thromboembolic phenomena – fingertip necrosis; cardiac source, arterial source, aneurysm (subclavian or axillary arteries), infection, hypercoagulable state AD 138:1296-1298, 2002 Thrombosis – penile gangrene Rook p.3194, 1998, Sixth Edition Thrombosis of large vessels Thrombotic thrombocytopenic purpura Urticarial vasculitis Clin Rev Allergy Immunol 23:201-216, 2002 Vasculitis – large and/or small vessel – leukocytoclastic vasculitis NEJM 352:700-707, 2005; AD 134:309-315, 1998; Rook p.2178, 1998, Sixth Edition; urticarial vasculitis AD 134:231-236, 1998 Vasoconstriction Venous stasis ulceration (chronic venous insufficiency) – medial lower leg and medial malleolus NEJM 352:700-707, 2005; AD 133:1231-1234, 1997; Semin Dermatol 12:66-71, 1993; with subcutaneous calcification J Derm Surg Oncol 16:450-452, 1990 Venous limb gangrene (phlegmasia cerulea dolens) – during warfarin treatment of cancer-associated deep venous thrombosis; due to severe depletion of protein C and failure to reduce thrombin generation Ann Intern Med 135:589-593, 2001 Congenital Volkmann ischemic contracture (neonatal compartment syndrome) – upper extremity circumferential contracture from wrist to elbow; necrosis, cyanosis, edema, eschar, bullae, purpura; irregular border with central white ischemic tissue with formation of bullae, edema, or spotted bluish color with necrosis, a reticulated eschar or whorled pattern with contracture of arm; differentiate from necrotizing fasciitis, congenital varicella, neonatal gangrene, aplasia cutis congenital, amniotic band syndrome, subcutaneous fat necrosis, epidermolysis bullosa BJD 150:357-363, 2004 Wegener’s granulomatosis – cutaneous necrosis NEJM 352:700-707, 2005; papulonecrotic lesions JAAD 48:311-340, 2003; Cutis 64:183-186, 1999; JAAD 31:615-622, 1994; necrotic penile ulcers Clin Rheumatol 17:239-241, 1998; JAAD 31:605-612, 1994; AD 130:1311-1316, 1994; genital and perineal necrosis Am J Med 108:680-681, 2000

African tick bite fever (Rickettsia africae) – hemorrhagic pustule, purpuric papules; transmitted by Amblyomma ticks – high fever, arthralgia, myalgia, fatigue, rash in 2-3 days, with eschar, maculopapules, vesicles, and pustules JAAD 48:S18-19, 2003

Anthrax Antiphospholipid antibody syndrome Aspergillosis Boutonneuse fever Brown recluse spider bite Calciphylaxis Capnocytophaga canimorsus sepsis – dog and cat bites; necrosis with eschar; cellulitis Cutis 60:95-97, 1997; JAAD 33:1019-1029, 1995 Coumadin necrosis Ecthyma Ecthyma gangrenosum Fusariosis Glanders Heparin necrosis Herpes simplex virus Milker’s nodule Mucormycosis Necrotizing fasciitis Orf Phagedenic ulcer Queensland tick typhus Rat bite fever Rickettsial pox Scrub typhus Siberian typhus Tularemia

Epidermal nevus syndrome Ped Derm 6:316-320, 1989 Limb hypoplasia J Pediatr 120:906-911, 1992 Linear nevus sebaceus syndrome Malformations associated with congenital nevi – spinal cord, vascular, neurocutaneous melanosis Melanocytic nevi along Blaschko’s lines Acta DV 78:378-380, 1998 Mills syndrome – giant congenital nevus and chronic progressive ascending hemiparesis Ital J Neurol Sci 13:259-263, 1992 Multiple hamartoma syndrome with congenital melanocytic nevus, epidermal nevi, vascular malformations, aplasia cutis congenita of the scalp, cartilage hamartoma, lipodermoid fibroma of the conjunctiva, and intracranial malformation Ped Derm 3:219-225, 1986 NAME/LAMB (Carney) syndrome Curr Prob Derm VII:143-198, 1995 Neurocutaneous melanosis JAAD 35:529-538,1996; JAAD 24:747-755. 1991 Neurofibromatosis Type I Neurology 35 (suppl 1):194, 1985 Nevi spili along Blaschko’s lines Acta DV 78:378-380, 1998

Noonan syndrome JAAD 46:161-183, 2002; Curr Prob Derm VII:143-198, 1995 Occult spinal dysraphism/tethered cord Semin Perinatol 7:253-256, 1983 Phakomatosis pigmentovascularis – pigmented nevus, vascular nevus, nevus spilus JAAD 53:536-539, 2005; nevus flammeus; blaschko-esque non-epidermolytic epidermal nevus, speckled lentiginous nevus with contralateral organoid nevus Clin Exp Dermatol 25:51-54, 2000; Ped Derm 16:25-30, 1999; Ped Derm 15:321-323, 1998; Ped Derm 13:33-35, 1996 Phakomatosis pigmentokeratotica – coexistence of an organoid nevus and a popular speckled lentiginous nevus Eur J Dermatol 10:190-194, 2000; Ped Derm 15:321-323, 1998; AD 134:333-337, 1998 Premature aging syndrome – Mulvihill-Smith progeria-like syndrome – multiple congenital melanocytic nevi, freckles, blue nevi, short stature, unusual birdlike facies, lack of facial subcutaneous tissue, xerosis, telangiectasias, thin skin, fine silky hair, premature aging, low birth weight, hypodontia, high-pitched voice, mental retardation, sensorineural hearing loss, hepatomegaly, microcephaly, immunodeficiency with chronic infections, progeroid, conjunctivitis, delayed puberty Am J Med Genet 69:56-64, 1997; J Med Genet 31:707-711, 1994; Am J Med Genet 45:597-600, 1993 Ring chromosome 7 syndrome – multiple congenital melanocytic nevi; CALMs

Addison’s disease – eruptive nevi JAAD 37:321-325, 1997 AIDS – eruptive nevi AD 135:397-401, 1989 Asthma melanodermica – prior to attack, diffuse darkening of skin and increase in size and number of nevi AD 78:210-213, 1958 Atypical mole syndrome (dysplastic nevus syndrome) JAAD 29:373-388, 1993; Recent Results Cancer Res 128:101-118, 1993; Cancer 46:1787-1794, 1980; J Med Genet 15:352-356, 1978 Cardio-facio-cutaneous syndrome (Noonan-like short stature syndrome) (NS) – xerosis/ichthyosis, eczematous dermatitis, growth failure, hyperkeratotic papules, ulerythema ophryogenes, seborrheic dermatitis, CALMs, nevi, keratosis pilaris, autosomal dominant, patchy or widespread ichthyosiform eruption, sparse curly short scalp hair and eyebrows and lashes, hemangiomas, acanthosis nigricans, congenital lymphedema of the hands, redundant skin of the hands, short stature, abnormal facies, cardiac defects JAAD 46:161-183, 2002; Ped Derm 17:231-234, 2000; JAAD 28:815-819, 1993; AD 129:46-47, 1993; JAAD 22:920-922, 1990; port wine stain Clin Genet 42:206-209, 1992 Chediak-Higashi syndrome Cheetah phenotype JAAD 48:707-713, 2003 Costello syndrome JAAD 32:904-907, 1995 Craniofacial anomalies, ocular findings, pigmented nevi, camptodactyly, skeletal changes – autosomal recessive Clin Dysmorphol 9:61-62, 2000 Drug-induced – immunosuppressive therapy in transplant recipients associated with increased number of nevi JAAD 28:51-53, 2001; JAAD 44:932-939, 2001; Dermatology 194:17-19, 1997; of the palms and soles JAAD 52:S96-100, 2005 Ectrodactyly, ectodermal dysplasia, and cleft palate syndrome (EEC syndrome) – increased numbers of nevi

Epidermal nevus syndrome – Spitz nevi Ped Derm 6:316-320, 1989 Epidermolysis bullosa – nevi associated with EB (‘EB nevi’) Ped Derm 22:338-343, 2005; JAAD 44:577-584, 2001; generalized atrophic benign EB (GABEB) (mitis) – non-lethal junctional – generalized blistering beginning in infancy; nevi or acquired macular pigmented lesions with irregular borders AD 122:704-710, 1986; GABEB – giant nevi at sites of blistering AD 132:145-150, 1996 Eruptive nevi without associations Erythema multiforme – eruptive nevi following erythema multiforme JAAD 37:337-339, 1997; JAAD 1:503-505, 1979 Facial nevi, anomalous cerebral venous return, and hydrocephalus Ann Neurol 3:316-318, 1978; J Neurosurg 45:20-25, 1976 Hypoplastic anemia with triphalangeal thumbs syndrome (Aase-Smith syndrome) Idiopathic scoliosis – related to number of acquired melanocytic nevi JAAD 45:35-43, 2001 Langer-Giedion syndrome (trichorhinophalangeal syndrome Type II) JAAD 29:373-388, 1993 Multiple deep penetrating nevi AD 139:1608-1610, 2003 Multiple schwannomas, multiple nevi, and multiple vaginal leiomyomas – autosomal dominant Am J Med Genet 78:76-81, 1998 Mustard gas – eruptive nevi JAAD 40:646-647, 1999 Neurologic syndromes – eruptive nevi after loss of consciousness or seizures BJD 92:207-211, 1975 Noonan’s syndrome – webbed neck, short stature, malformed ears, nevi, keloids, transient lymphedema, ulerythema ophyrogenes, keratosis follicularis spinulosa decalvans JAAD 46:161-183, 2002; Rook p.3016, 1998, Sixth Edition; J Med Genet 24:9-13, 1987 Phacomatosis pigmentovascularis type IIIb – nevus spilus AD 125:1284-1285, 1989 Renal transplantation – eruptive atypical nevi in renal transplantation Stevens-Johnson syndrome – eruptive nevi JAAD 37:337-339, 1997 Sunburn, blistering – eruptive nevi Telangiectasia macularis eruptiva perstans – mastocytosis – nevus-like appearance Toxic epidermal necrolysis – eruptive nevi Tricho-odonto-onychodysplasia syndrome – multiple melanocytic nevi, freckles, generalized hypotrichosis, parietal alopecia, brittle nails, xerosis,supernumerary nipples, palmoplantar hyperkeratosis, enamel hypoplasia, deficient frontoparietal bone JAAD 29:373-388, 1993; Am J Med Genet 15:67-70, 1983 Turner’s syndrome (XO in 80%) – multiple nevi; peripheral edema at birth which resolves by age 2; redundant neck skin in newborn; small stature, broad shield-shaped chest with widely spaced nipples, arms show wide carrying angle, webbed neck, low posterior hairline, low misshapen ears, high arched palate, cutis laxa of neck and buttocks, short fourth and fifth metacarpals and metatarsals, hypoplastic nails, keloid formation, increased numbers of nevi; skeletal, cardiovascular, ocular abnormalities; increased pituitary gonadotropins with low estrogen levels JAAD 50:767-776, 2004; JAAD 46:161-183, 2002; JAAD 40:877-890, 1999 Xanthoma disseminatum – nevi-like appearance of xanthoma disseminatum AD 138:1207-1212, 1992

Baraitser syndrome – premature aging with short stature and pigmented nevi Crouzon syndrome Ped Derm 13:18-21, 1996 Ectrodactly-ectodermal dysplasia-cleft lip/palate (EEC) syndrome Epidermolysis bullosa – generalized atrophic benign EB (GABEB) (mitis) – non-lethal junctional – generalized blistering beginning in infancy; atrophic scarring; alopecia of scalp, eyebrows, eyelashes Dermatologica 152:72-86, 1976; nevi or acquired macular pigmented lesions with irregular borders AD 122:704-710, 1986; GABEB – giant nevi at sites of blistering AD 132:145-150, 1996 Familial atypical mole syndrome Bull Cancer 85:627-630, 1998 Familial multiple blue nevi Goeminne syndrome – X-linked incompletely dominant, multiple melanocytic nevi, multiple spontaneous keloids, basal cell carcinomas, varicose veins, muscular toricollis, renal dysplasia, cryptorchidism, urethral meatal stenosis, facial asymmetry, clinodactyly, scoliosis, dental caries JAAD 29:373-388, 1993; Curr Prob Derm VII:143-198, 1995 Jaffe-Campanacci syndrome Curr Prob Derm VII:143-198, 1995 Kuskokwin syndrome – autosomal recessive, multiple melanocytic nevi, joint contractures, muscle atrophy, decreased corneal reflexes, skeletal abnormalities Curr Prob Derm VII:143-198, 1995 LEOPARD syndrome Leukemia – eruptive nevi with chronic myelogenous leukemia JAAD 35:326-329, 1996 Moynahan syndrome Proc R Soc Med 55:959-960, 1962 Mulvihill-Smith syndrome (premature aging, microcephaly, unusual facies, multiple nevi, mental retardation) Birth Defects 11:368-371, 1975 Neurofibroma resembling congenital melanocytic nevus JAAD 20:358-362, 1989 Nevoid basal cell carcinoma syndrome Nevus sebaceus syndrome Syndromes of the Head and Neck, p.362, 1990 Noonan’s syndrome – short stature, webbed neck, shield chest, lymphedema, dystrophic nails, keloids, and increased numbers of nevi Ped Derm 11:120-124, 1994 Phakomatosis pigmentokeratotica – speckled lentiginous nevus in checkerboard pattern AD 134:333-337, 1998 Phakomatosis pigmentovascularis – nevus spilus in phacomatosis pigmentovasularis type IIIb AD 125:1284-1285, 1989 Progeroid short stature with pigmented nevi J Med Genet 25:53-56, 1988 Proteus syndrome – partial gigantism of the hands and/or feet, nevi, hemihypertrophy, subcutaneous tumors, macrocephaly, other skull anomalies, accelerated growth Eur J Pediatr 140:5-12, 1983 Short stature, premature aging, pigmented nevi J Med Genet 25:53-56, 1988 Tay syndrome – lentigines Tricho-odonto-onychial dysplasia Tricho-onychodysplasia syndrome Trisomy 22 – Turner-like changes

Turner’s syndrome – webbed neck, hypoplastic nails, keloids and hypertrophic scars, xerosis, seborrheic dermatitis, abnormal dermatoglyphics, increased numbers of nevi, sexual infantilism, short stature, cubitus valgus Ped Derm 11:120-124, 1994; JAAD 36:1002-1004, 1996; NEJM 335:1749-1754, 1996; Curr Prob Derm VII:143-198, 1995

NIPPLE DERMATITIS JAAD 43:733-751, 2000

Allergic contact dermatitis Contact Dermatitis 45:44-45, 2001; Contact Dermatitis 33:440-441, 1995; Contact Dermatitis 24:139-140, 1991 Bullous pemphigoid Chronic granulomatous disease DiGeorge’s syndrome Cutis 45:455-459, 1990 Pemphigus foliaceus Pemphigus vulgaris Severe combined immunodeficiency Ped Derm 9:49-51, 1992

Heparin JAAD 20:1130-1132, 1989

Irritant contact dermatitis Hair sinus

Bejel – endemic syphilis Candidiasis Herpes simplex virus – eczema herpeticum Histoplasmosis JAAD 25:418-422, 1991 HIV infection in children JAAD 22:1223-1231, 1990 Molluscum contagiosum Mycobacterium tuberculosis – tuberculous mastitis, lupus vulgaris Pathologee 18:67-70, 1997 Scabies Rook p.1460-1461, 1998, Sixth Edition Staphylococcal infection of the nipple (inflammatory plaques) JAAD 20:932-934, 1989 Syphilis – secondary Subareolar abscess Tinea corporis Tinea versicolor Verruca vulgaris

Mucopolysaccharidosis

Hidradenitis suppurativa Neutrophilic eccrine hidradenitis Periductal mastitis – nipple discharge Ghatan p.51, 2002, Second Edition Pyoderma gangrenosum Superficial granulomatous pyoderma

Prolidase deficiency JAAD 29:819-821, 1993

Adenocarcinoma Basal cell carcinoma – crusted dermatitis JAAD 22:207-210, 1990 Blue nevus Bowen’s disease Derm Surg 27:971-974, 2001 Breast carcinoma – direct spread from underlying breast carcinoma; primary inflammatory breast carcinoma; nipple discharge Clear cell acanthoma BJD 141:950-951, 1999 Epidermal nevus Keratoacanthoma Lentigo maligna Cutis 40:357-359, 1987 Lymphoma – cutaneous T-cell lymphoma; cutaneous T-cell lymphoma presenting as hyperkeratosis areolae mammae JAAD 41:274-276, 1999 Melanoma Metastases – carcinoma erysipelatoides Nevus, melanocytic Paget’s disease of the breast (nipple) Rook p.1677-1678,2709, 1998, Sixth Edition; Br J Clin Pract 41:694-696, 1987; Dermatologica 170:170-179, 1985; Surg Gynecol Obstet 123:1010-1014, 1966; in male J Cut Surg Med 4:208-212, 2000 Sebaceous hyperplasia Cutis 58:63-64, 1996 Seborrheic keratoses Verrucous acanthoma Wart

Acanthosis of nipple Acanthosis nigricans/pseudoacanthosis nigricans Apocrine chromhidrosis – black dot of nipple Ped Derm 12:48-50, 1995 Atopic dermatitis Ped Derm 22:64-66, 2005; Trans St John’s Hosp Dermatol Soc 58:98-99, 1972 Darier’s disease – erosions JAAD 23:893-897, 1990 Erosive adenomatosis of the nipple – blood-stained or serous discharge; enlarged nipple; eroded, crusted, dermatitis; papule on nipple JAAD 40:834-837, 1999; Cutis 59:91-92, 1997; JAAD 12:707-715, 1985; papillary adenoma of the nipple Plast Reconstr Surg 90:1077-1078, 1992 Erythema craquele Florid papillomatosis of the nipple Ghatan p.51, 2002, Second Edition

Fox-Fordyce disease Hailey-Hailey disease Hyperkeratosis of the nipple (hyperkeratosis areolae mammae) JAAD 41:274-276, 1999; Australas J Dermatol 40:220-222, 1999; JAAD 13:596-598, 1985 Idiopathic dermatitis of the nipple Trans St John’s Hosp Dermatol Soc 58:98-99, 1972 Lichen sclerosus et atrophicus BJD 129:748-749, 1993 Lichen simplex chronicus Mamillary fissure Mammary duct ectasia JAAD 40:834-837, 1999 Milium Nummular dermatitis Psoriasiform dermatitis Psoriasis Seborrheic dermatitis Subareolar duct papillomatosis

Factitial dermatitis

Netherton’s syndrome Neurofibromatosis

Breast feeding (lactation mastitis) – edema, erythema and tenderness JAMA 289:1609-1612, 2003 Guitar nipple JAAD 22:657-663, 1990 Jogger’s nipple Am J Ind Med 8:403-413, 1985; NEJM 297:1127, 1977 Nipple ring Radiation dermatitis Surf rider’s dermatitis Contact Dermatitis 32:247, 1995

Wegener’s granulomatosis

Pemphigus foliaceus

Absent or rudimentary nipples with lumpy scalp and unusual pinnae (Finlay syndrome) – autosomal dominant Genet Couns 2:233-236, 1991; BJD 99:423-430, 1978 Adnexal polyp of neonatal skin BJD 92:659-662, 1975 Carbohydrate-deficient glycoprotein syndrome – inverted nipples; emaciated appearance; lipoatrophy over buttocks;

lipoatrophic streaks extend down legs; high nasal bridge, prominent jaw, large ears, fat over suprapubic area and labia majora, fat pads over buttocks; hypotonia Textbook of Neonatal Dermatology, p.432, 2001 Circumareolar telangiectasia AD 126:1656, 1990 Galactorrhea of newborn – witches’ milk Mamillary fistula Pearls (milia) in newborn of areolae, scrotum, and labia majora of newborn Rook p.452, 1998, Sixth Edition Rudimentary nipples Humangenetik 15:268-269, 1972 Supernumery nipples (accessory nipple or nipple nevus) Textbook of Neonatal Dermatology, p.118, 2001; Cutis 62:235-237, 1998

Diethylstilbestrol – florid papillomatosis after diethylstilbestrol therapy in males Nipple priapism JAMA 258:3122, 1987

Nipple rings Rook p.3158, 1998, Sixth Edition

Candida albicans – lactation Aust NZ J Obstet Gynaecol 31:378-380, 1991 Herpes simplex Tyring p.83, 2002 Lyme disease (Borrelia burgdorferi ) – lymphocytoma cutis; bluish-red plaque of nipple or areola JAAD 49:363-392, 2003; JAAD 47:530-534, 2002; Cutis 66:243-246, 2000; JAAD 38:877-905, 1998 Molluscum contagiosum Mycobacterium abscessus – subareolar abscess due to nipple piercing Clin Inf Dis 33:131-134, 2001 Mycobacterium tuberculosis – lupus vulgaris Cutis 53:246-248, 1994 Scabies Rook p.1460-1461, 1998, Sixth Edition Syphilis – primary chancre; secondary Rook p.1244, 1998, Sixth Edition

Mucinosis of the areolae – manifestation of CTCL Clin Exp Dermatol 21:374-376, 1996

Lactation mastitis JAMA 289:1609-1612, 2003

Addison’s disease – hyperpigmentation of nipples Rook p.1779, 1998, Sixth Edition Androgen excess – hyperpigmentation of areolae, axillae, external genitalia, perineum Ghatan p.165, 2002, Second Edition Nipple retraction of menopause Br Med J 309:797-800, 1994 Pregnancy – hyperpigmentation of nipples Rook p.1780,3149, 1998, Sixth Edition

Basal cell carcinoma – red plaque Derm Surg 27:971-974, 2001; Cutis 54:85-92, 1994; including pigmented basal cell carcinoma (black nipple) AD 125:536-539, 1989 Blue nevus Breast cancer – female or male; puckered nipple Carcinoma of the breast (primary), metastatic to nipple – cellulitis-like appearance of primary inflammatory breast carcinoma JAAD 43:733-751, 2000; female or male breast carcinoma – puckered nipple Dermatofibroma – atypical polypoid dermatofibroma JAAD 24:561-565, 1991 Epidermal nevus Paris Med 28:63-66, 1938 Epidermoid cyst Fox-Fordyce disease Rook p.3162, 1998, Sixth Edition Granular cell myoblastoma – nipple-like lesion AD 121:927-932, 1985 Hidradenoma papilliferum Hautarzt 19:101-109, 1968 Hidradenoma Leiomyomas AD 127:571-576, 1991 Leiomyosarcoma – blue-black; also red, brown, yellow or hypopigmented JAAD 46:477-490, 2002 Lymphoma – cutaneous T-cell lymphoma mimicking hyperkeratosis of the nipple (hyperkeratosis areolae mammae) JAAD 41:274-276, 1999 Lymphocytoma cutis Melanoma – primary, metastatic, amelanotic Milium Neurofibromas Rook p.3162, 1998, Sixth Edition Nevi, melanocytic Nevoid hyperkeratosis of the nipple JAAD 46:414-418, 2002; BJD 142:382-384, 2000; JAAD 41:325-326, 1999 Paget’s disease of the breast (nipple) Rook p.1677-1678,2709, 1998, Sixth Edition; Dermatologica 170:170-179, 1985; Surg Gynecol Obstet 123:1010-1014, 1966 Prostatic carcinoma metastases JAAD 18:391-393, 1988 Sebaceous hyperplasia of areola JAAD 13:867-868, 1985 Seborrheic keratoses Rook p.3161, 1998, Sixth Edition Steatocystoma multiplex Syringocystadenoma papilliferum Syringomas Syringomatous adenomas of the nipple

Montgomery’s tubercles JAAD 10:929-940, 1984; BJD 86:126-133, 1972

Acanthosis nigricans JAAD 52:529-530, 2005; JAAD 31:1-19, 1994 Epidermolytic hyperkeratosis – verrucous plaques of nipples Rook p.1506, 1998, Sixth Edition Erosive adenomatosis (papillary adenomatosis) of the nipple – blood-stained or serous discharge; enlarged nipple; eroded nipple; erythema, ulcer, crusted dermatitis, granular appearance, papule on nipple JAAD 47:578-580, 2002; JAAD 43:733-751, 2000; superficial papillary adenomatosis of the nipple JAAD 33:871-875, 1995; JAAD 12:707-715, 1985

Florid papillomatosis of the nipple Ghatan p.51, 2002, Second Edition Fox-Fordyce disease Rook p.2002, 1998, Sixth Edition Hyperkeratosis of the nipple and areola (hyperkeratosis areolae mammae) AD 137:1327-1328, 2001; JAAD 41:274-276, 1999; Eur J Dermatol 8:131-132, 1998; AD 126:687, 1990; JAAD 13:596-598, 1985; estrogen-induced Cutis 26:95-96, 1980; associated with CTCL JAAD 32:124-125, 1995; Int J Derm 29:519-520, 1990; ichthyosis, ichthyosiform erythroderma, acanthosis nigricans, Darier’s disease Rook p.3157, 1998, Sixth Edition Inverted nipple Ann Plast Surg 25:457-460, 1990 Lichen sclerosus et atrophicus Lichen simplex chronicus Mamillary fistula Ghatan p.51, 2002, Second Edition Morphea Rook p.3162, 1998, Sixth Edition Psoriasis Rook p.3162, 1998, Sixth Edition Sebaceous glands – ectopic sebaceous glands (Fordyce spots) – areola of nipple J Dermatol 21:524-526, 1994 Vitiligo JAAD 38:647-666, 1998

Factitial dermatitis

Ablepharon macrostomia – absent eyelids, ectropion, abnormal ears, rudimentary nipples, dry redundant skin, macrostomia, ambiguous genitalia Hum Genet 97:532-536, 1996 ACC with nipple and breast hypoplasia, nail dysplasia, delayed dentition Am J Med Genet 14:381-384, 1983 Bannayan-Riley-Ruvalcaba-Zonana syndrome (PTEN phosphatase and tensin homolog hamartoma) – accessory nipple, dolicocephaly, frontal bossing, macrocephaly, ocular hypertelorism, long philtrum, thin upper lip, broad mouth, relative micrognathia, lipomas, penile or vulvar lentigines, facial verruca-like or acanthosis nigricans-like papules, multiple acrochordons, angiokeratomas, transverse palmar crease, syndactyly, brachydactyly, vascular malformations, arteriovenous malformations, lymphangiokeratoma, goiter, hamartomatous intestinal polyposis JAAD 53:639-643, 2005 Hereditary acrolabial telangiectasia – blue lips, blue nails, blue nipples, telangiectasia of the chest, elbows, knees, dorsa of hands, varicosities of the legs, migraine headaches AD 115:474-478, 1979 Birt-Hogg-Dube – trichodiscomas JAAD 16:452-457, 1987 Carney complex – cutaneous myxomas of the nipples (papules) JAAD 43:377-379, 2000; Cutis 62:275-280, 1998; Gardner’s syndrome – epidermoid cysts Finlay-Marks syndrome (scalp-ear-nipple syndrome) – nipple or breast hypoplasia or aplasia, aplasia cutis congenita of scalp, cleft lip/palate, cardiac malformations, polydactyly, narrow convex nails Bolognia p.924, 2003 Hutchinson-Gilford syndrome (progeria) – hypoplastic nipples Am J Med Genet 82:242-248, 1999; J Pediatr 80:697-724, 1972 Incontinentia pigmenti – hyperpigmented nipples; supernumerary nipples; nipple hypoplasia AD 139:1163-1170, 2003; JAAD 47:169-187, 2002 Johanson-Blizzard syndrome – autosomal recessive; growth retardation, microcephaly, ACC of scalp, sparse hair, hypoplastic ala nasi, CALMs, hypoplastic nipples and areolae, hypothyroidism, sensorineural deafness Clin Genet 14:247-250, 1978 Keratosis-ichthysosis-deafness (KID) syndrome – acanthosis nigricans-like change of the nipple AD 123:777-782, 1987; hypoplasia of nipples Ped Derm 19:513-516, 2002

Lumpy scalp, odd ears, and rudimentary nipples BJD 99:423-430, 1978 Neurofibromatosis Rook p.3162, 1998, Sixth Edition NAME/LAMB syndrome AD 122:790-798, 1986 Oculo-osteocutaneous syndrome – sparse, fair hair, limb and digit abnormalities, hypoplastic nipples, abnormal genitalia Ped Derm 19:226, 2002 Rapp-Hodgkin hypohidrotic ectodermal dysplasia – autosomal dominant; alopecia of wide area of scalp in frontal to crown area, short eyebrows and eyelashes, coarse wiry sparse hypopigmented scalp hair, sparse body hair, scalp dermatitis, ankyloblepharon, syndactyly, nipple anomalies, cleft lip and/or palate; nails narrow and dystrophic, small stature, hypospadius, conical teeth and anodontia or hypodontia; distinctive facies, short stature JAAD 53:729-735, 2005; Ped Derm 7:126-131, 1990; J Med Genet 15:269-272, 1968 Rubinstein-Taybi syndrome – supernumerary nipples JAAD 46:159, 2002 Scalp-ear-nipple syndrome – autosomal dominant; aplasia cutis congenita of the scalp, irregularly shaped pinna, hypoplastic nipple, widely spaced teeth, partial syndactyly Am J Med Genet 50:247-250, 1994 Simpson-Golabi-Behmel syndrome – X-linked; increased growth, accessory nipples, coarse facies, polydactyly, midline defects, mental retardation Am J Med Genet 46:606-607, 1993 Turner’s syndrome – shield chest with widely spaced nipples JAAD 50:767-776, 2004

Guitar nipple – cystic swelling at base of nipple Br Med J 2:226, 1974

Acrocyanosis – blue nipples JAAD S207-208, 2001 Peripheral symmetric gangrene Raynaud’s phenomenon Br Med J 314:644-645, 1997 Thrombosed angioma

NODULES, CONGENITAL Ped Derm 9:301, 1991 Adipose plantar nodules BJD 142:1262-1264, 2000 Arteriovenous malformation JAAD 46:934-941, 2002 Benign and/or disseminated neonatal hemangiomatosis JAAD 24:816-818, 1991; Ped Derm 8:140-146, 1991 Blueberry muffin lesions of congenital infections or ABO/Rh incompatibility Ann DV 125:199-201, 1998 Branchial sinus and/or cyst Cartilaginous rest of the neck – nodule over medial clavicle Cutis 58:293-294, 1996; AD 127:1309-1310, 1991 Cephalocoele – includes encephalocoele, meningocoele (rudimentary meningocoele), meningoencephalocoele, meningomyelocoele; blue nodule with overlying hypertrichosis JAAD 46:934-941, 2002; AD 137:45-50, 2001 Cephalohematoma (cephalohematoma deformans) – blood between outer table of skull and periosteum; fixed Ped Clin North Am 6:1151-1160, 1993 Cervical thyroid

Congenital self-healing Langerhans cell histiocytosis (Hashimoto-Pritzker disease) – multiple congenital red-brown nodules Ped Derm 17:322-324, 2000 Dermoid cyst AD 135:463-468, 1999 Eccrine angiomatous hamartoma JAAD 47:429-435, 2002; Ped Derm 14:401-402, 1997; Ped Derm 13:139-142, 1996; skin-colored nodule with blue papules JAAD 41:109-111, 1999 Embryonal rhabdomyosarcoma – orbit, nasopharynx, nose Rook p.2369, 1998, Sixth Edition Encephalocoele Ewing’s sarcoma Fibromatosis – congenital generalized fibromatosis Ped Derm 8:306-309, 1991; AD 122:89-94, 1986; JAAD 10:365-371, 1984 Fibrosarcoma, neonatal JAAD 50:S23-25, 2004 Fibrous hamartoma of infancy – benign, single, soft to firm ill-defined subcutaneous mass present at birth or before 2 years of age; more common in boys, freely movable, most commonly located on the shoulder, arm or axillary region Ped Derm 13:171-172, 1996 Glomangioma Ped Derm 12:242-244, 1995 Glomangiomyoma – congenital multiple plaque-like glomangiomyoma Am J Dermatopathol 21:454-457, 1999 Gonorrhea – newborn with gonococcal scalp abscess South Med J 73:396-397, 1980; Am J Obstet Gynecol 127:437-438, 1977 Hamartoma with ectopic meningothelial elements – simulates angiosarcoma Am J Surg Pathol 14:1-11, 1990 Hemangiomas JAAD 46:934-941, 2002 Hemangiopericytoma J Bone Joint Surg Br 83:269-272, 2001 Heterotopic brain tissue (heterotopic meningeal nodules) – blue-red cystic mass with overlying alopecia JAAD 46:934-941, 2002; bald cyst of scalp with surrounding hypertrichosis AD 131:731, 1995; JAAD 28:1015, 1993; BJD 129:183-185, 1993; AD 125:1253-1256, 1989; cyst with collar of hair (heterotopic meningeal nodules) JAAD 28:1015-1017, 1993; AD 123:1253-1256, 1989 Histiocytosis Congenital immune deficiencies with cutaneous granulomas Ann DV 122:501-506, 1995 Infantile myofibromatosis Pediatrics 104:113-115, 1999; solitary nodule S Afr Med J 64:590-591, 1983 Juvenile xanthogranuloma – giant congenital form Ann DV 122:678-681, 1995 Kaposiform hemangioendothelioma Am J Surg Pathol 17:321-328, 1993 Leiomyoma Ped Derm 3:158-160, 1986 Leukemia – neonatal monoblastic leukemia Ann DV 126:157-159, 1999; lymphoblastic leukemia JAAD 34:375-378, 1996; AD 129:1301-1306, 1993; neonatal myelomonocytic leukemia Lipoblastomatosis – congenital lipoblastomatosis Ped Derm 15:210-213, 1998 Lipoma – vulvar AD 118:447, 1982 Lymphoma Malignant hemangiopericytoma West Afr J Med 19:317-318, 2000 Malignant rhabdoid tumor Arch Pathol Lab Med 122:1099-1102, 1998 Mastocytosis Melanocytic nevi with nodules J Dermatol 23:828-831, 1996 Meningioma – scalp nodule Eur J Pediatr Surg 10:387-389, 2000 Meningocoele Midline raphe cyst of the scrotum

Nasal glioma – red or bluish nodules; resemble hemangiomas, nasal encephalocoele, meningoencephalocoele, extracranial meningioma, dermoid cyst, larrimal duct cyst, neuroblastoma, rhabdomyosarcoma Ear Nose Throat J 80:410-411, 2001; Rook p.603, 1998, Sixth Edition; Arch Otolaryngol 107:550-554, 1981 Nerve sheath myxoma (neurothekoma) of the tongue Oral Surg Oral Med Oral Pathol Oral Radiol Endod 90:74-77, 2000 Neuroblastoma J Formos Med Assoc 90:422-425, 1991 Neurocristic cutaneous hamartoma Mod Pathol 11:573-578, 1998; malignant melanotic neurocristic tumors arising in neurocristic hamartomas Am J Surg Pathol 20:665-677, 1996 Neurofibroma Peripheral primitive neuroectodermal tumor Med Pediatr Oncol 30:357-363, 1998 Pyogenic granuloma – presenting as congenital epulis Arch Pediatr Adolesc Med 154:603-605, 2000 Pyramidal lobe of thyroid gland Rapidly involuting congenital hemangioma (RICH) – violaceous large mass with central telangiectasia JAAD 50:875-882, 2004 Rhabdomyosarcoma Sinus pericranii – alopecic red nodule of scalp JAAD 46:934-941, 2002 Subepicranial hygromas JAAD 46:934-941, 2002 Venous cavernoma (venous malformation) JAAD 46:934-941, 2002; Zentralbl Neurochir 59:274-277, 1998 Wattle; cutaneous cervical tag AD 121:22-23, 1985 Wilms’ tumor

Behçet’s disease J Rheumatol 25:2469-2472, 1998 Chronic granulomatous disease – chilblains JAAD 36:899-907, 1997; X-linked chronic granulomatous disease – photosensitivity, chilblain lupus of fingertips and toes Ped Derm 3:376-379, 1986 Common variable immunodeficiency (Gottron-like papules) – granulomas presenting as acral red papules and plaques with central scaling, scarring, atrophy, ulceration Cutis 52:221-222, 1993 Rheumatoid nodule J Dermatol 24:798, 1997; plantar nodule JAAD 53:191-209, 2005

Congenital infantile digital fibromatosis Ped Derm 19:370-371, 2002 Congenital pedal papules Textbook of Neonatal Dermatology, p.429, 2001

Dupuytren’s contracture – in children Br J Plast Surg 41:313-315, 1988

Hydantoin – thickening of the heel pad due to long term hydantoin therapy Am J Roentgenol Radium Ther Nucl Med 124:52-56, 1975 Neutrophilic eccrine hidradenitis, chemotherapy-induced

Foreign body granuloma Sea urchin spine – plantar nodule

Alternariosis – red nodule of foot Clin Inf Dis 30:13,174-175, 2000 Anthrax Arthropod bites Bacillary angiomatosis Tyring p.228, 2002 Brucellosis Cat scratch fever Chromomycosis JAAD 32:390-392, 1995 Coccidioidomycosis – granuloma of foot Ghatan p.62, 2002, Second Edition Cryptococcosis Diphtheria Ecthyma Herpes simplex, chronic Herpes zoster, chronic Insect bites – plantar nodule Ped Derm 15:97-102, 1998 Leishmaniasis – post-kala-azar dermal leishmaniasis Leprosy Int J Lepr Other Mycobact Dis 68:272-276, 2001 Mycetoma JAAD 32:311-315, 1995; Cutis 49:107-110, 1992; Australas J Dermatol 31:33-36, 1990; JAAD 6:107-111, 1982; Sabouraudia 18:91-95, 1980; AD 99:215-225, 1969 Mycobacterium avium-intracellulare AD 124:1545-1549, 1988 Mycobacterium chelonei JAAD 28:809-810, 812-813, 1993 Mycobacterium kansasii JAAD 24:208-215, 1991 Mycobacterium marinum – nodule or papule of hands, elbows, knees becomes crusted ulcer or abscess; or verrucous papule; sporotrichoid; rarely widespread lesions Br Med J 300:1069-1070, 1990; AD 122:698-703, 1986; J Hyg 94:135-149, 1985 Mycobacterium tuberculosis – lupus vulgaris Int J Derm 40:336-339, 2001 Nocardiosis Pseudomonas hot tub folliculitis – palmoplantar red nodules; Pseudomonas hot-foot syndrome – 1-2-cm plantar nodules; spontaneous resolution in 14 days NEJM 345:335-338, 2001 Rocky Mountain spotted fever – palmoplantar red nodules Scabies, nodular J Cutan Pathol 19:124-127, 1992; crusted (Norwegian) scabies presenting with hyperkeratotic nodules of the soles AD 134:1019-1024, 1998 Septic emboli Trichophyton rubrum – invasive in immunocompromised host JAAD 39:379-380, 1998; mimicking Kaposi’s sarcoma Int J Dermatol 18:751-752, 1979 Tungiasis (Tunga penetrans) (toe-tip or subungual nodule) – crusted or ulcerated Caputo p.164, 2000; Acta Dermatovenerol (Stockh) 76:495, 1996; JAAD 20:941-944, 1989; AD 124:429-434, 1988 Warts, including human papillomavirus type 60-associated plantar wart; myrmecia (deep periungual or plantar warts) BMJ 1:912-915, 1951

Amyloidosis Cutis 59:142, 1997; nodular amyloidoisis of the toe AD 139:1157-1159, 2003 Juvenile xanthogranuloma of sole Ped Derm 15:203-206, 1998

Erythema multiforme – plantar nodules Ped Derm 15:97-102, 1998 Erythema nodosum – plantar nodules Dermatology 199:190, 1999; JAAD 40:654-655, 1994 Neutrophilic eccrine hidradenitis of the palms and soles – idiopathic in children Eur J Pediatr 160:189-191, 2001; AD 134:76-79, 1998; J Cutan Pathol 21:289-296, 1994; idiopathic recurrent palmoplantar eccrine hidradenitis – often post-traumatic Ped Derm 21:466-468, 2004; BJD 142:1048-1050, 2000 Panniculitis – due to blind loop syndrome JAAD 37:824-827, 1997; plantar nodule Ped Derm 15:97-102, 1998 Relapsing eosinophilic perimyositis – fever, fatigue, and episodic muscle swelling; erythema over swollen muscles BJD 133:109-114, 1995 Sarcoid – painful palmoplantar nodules Med Cutan Ibero Lat Am 15:384-386, 1987

Calcinosis cutis – tumoral calcinosis; calcified cutaneous nodules – heels of children due to heel sticks as a neonate Ped Derm 18:138-140, 2001 Gout Cutis 48:445-451, 1991; Ann Rheum Dis 29:461-468, 1970 Thyroid acropachy Xanthomas – tendinous, tuberous xanthomas

Adipose plantar nodules (congenital) BJD 142:1262-1264, 2000 Angioleiomyoma J Foot Surg 31:372-377, 1992 Angiolipoma J Foot Surg 31:17-24, 1992 Atypical fibroxanthoma Childhood fibrous hamartoma – plantar nodule Ped Derm 17:429-431, 2000 Chondroid syringoma J Am Podiatr Med Assoc 79:563-565, 1989; malignant chondroid syringoma of the foot Am J Clin Oncol 23:227-232, 2000 Clear cell hidradenoma (nodular hidradenoma, eccrine sweat gland adenoma of the clear cell type, solid cystic hidradenoma, eccrine acrospiroma) Dermatol Surg 26:685-686, 2000 Combined type blue nevus Ped Derm 14:358-360, 1994 Connective tissue nevus, including eruptive collagenoma Dermatofibroma – dorsum of foot; plantar nodule Ped Derm 21:506-507, 2004 Digital fibrous tumor of childhood – toe nodule AD 131:1195-1198, 1995 Eccrine acrospiroma Eccrine angiomatous hamartoma – toes, fingers, palms and soles – skin-colored to blue Cutis 71:449-455, 2003; JAAD 47:429-435, 2002; Ped Derm 13:139-142, 1996; JAAD 37:523-549, 1997; Ped Derm 14:401-402, 1997; Ped Derm 18:117-119, 2001; Ped Derm 14:401-402, 1997; skin-colored nodule with blue papules JAAD 41:109-111, 1999 Eccrine poroma – plantar red nodule Rook p.1706-1707, 1998, Sixth Edition; AD 74:511-521, 1956 Eccrine syringofibroadenoma (acrosyringeal hamartoma) JAAD 41:650-651, 1999 Elastoma – isolated, Buschke-Ollendorf Embryonal rhabdomyosarcoma – plantar nodule Ped Derm 21:506-507, 2004 Enchondromas

Epidermoid cysts, plantar – HPV-60-related BJD 152:961-967, 2005 Fibrosarcoma, congenital – plantar nodule Ped Derm 21:506-507, 2004 Fibrous hamartoma of infancy – congenital plantar nodule Ped Derm 21:506-507, 2004 Fibrous histiocytoma, benign Cutis 46:223-226, 1990 Ganglion cyst Giant cell tumor of the tendon sheath J Bone Joint Surg Am 66:76-94, 1984 Infantile histiocytic nodules J R Soc Med 77 Suppl:19-21, 1984 Juvenile xanthogranuloma – brown nodule of sole with rim of hyperkeratosis Ped Derm 17:460-462, 2000 Kaposi’s sarcoma – classic type Rook p.1063,2358-2360, 1998, Sixth Edition; JAAD 38:143-175, 1998; Int J Dermatol 36:735-740, 1997; Dermatology 190:324-326, 1995 Keloids Leiomyoma Lipoma – plantar nodule Ped Derm 21:506-507, 2004 Lymphocytoma cutis Lymphoma, including cutaneous T-cell lymphoma Malignant fibrous histiocytoma, myxoid variant – papule or nodule of ankle JAAD 48:S39-40, 2003; JAAD 43:892, 2000; Caputo p.103, 2000 Malignant giant cell tumor of soft parts Am J Dermatopathol 11:197-201, 1989 Malignant melanoma of the soft parts (clear-cell sarcoma) – nodule of tendons of foot Cancer 65:367-374, 1990 Melanoma Derm Surg 27:591-593, 2001 Mesenchymal hamartoma J Dermatol 25:406-408, 1998 Myofibroma – plantar nodule Ped Derm 21:506-507, 2004 Nevus lipomatosis Neurilemmoma (schwannoma) – pink or vascular nodule of the foot Cutis 67:127-129, 2001 Neuromas Am J Podiatr Med Assoc 90:252-255, 2000 Nodular fasciitis Ped Derm 21:506-507, 2004 Plantar aponeurotic fibroma Ped Derm 17:429-431, 2000; anteromedial plantar fibromatosis of childhood Ped Derm 17:472-474, 2000 Plantar fibromatosis (Ledderhose’s disease) – red plantar nodule; painful; may ulcerate Cutis 68:219-222, 2001; Curr Prob Derm 8:137-188, 1996 Plexiform schwannoma of the foot Eur Radiol 9:1653-1655, 1999 Precalcaneal congenital fibrolipomatous hamartoma – benign anteromedial plantar nodule of childhood-a distinct form of plantar fibromatosis Ped Derm 17:429-431, 2000 Schwannoma (neurilemmoma) – vascular nodule of the foot Cutis 67:127-129, 2001 Spindle cell hemangioendothelioma hyperkeratotic nodules of soles BJD 142:1238-1239, 2000; J Dermatol 18:104-111, 1991 Squamous cell carcinoma Verrucous carcinoma (epithelioma cuniculatum)

Granuloma annulare – deep granuloma annulare Arch Pediatr 2:858-860, 1995 (Fr); perforating granuloma annulare – palmoplantar red nodule JAAD 32:126-127, 1995; congenital granuloma annulare Ped Derm 22:234-236, 2005 Lichen sclerosus et atrophicus – ankle nodule JAAD 31:817-818, 1994

Macrodactyly, primary – giant nodule of plantar surface Migratory angioedema – plantar nodule Ped Derm 15:97-102, 1998 Prurigo nodularis

Blue rubber bleb nevus syndrome – plantar blue nodules Ehlers-Danlos syndrome Familial cutaneous collagenoma Fibroblastic rheumatism J Rheumatol 25:2261-2266, 1998 Focal dermal hypoplasia Hemihyperplasia-multiple lipomatosis syndrome – extensive congenital vascular stain, compressible blue nodule, multiple subcutaneous nodules, hemihypertrophy, syndactyly, thickened but not cerebriform soles, dermatomyofibroma Soc Ped Derm Annual Meeting, July 2005; Am J Med Genet 130A:111-122, 2004; Am J Med Genet 79:311-318, 1998 Infantile digital fibromatosis J Dermatol 25:523-526, 1998 Multicentric reticulohistiocytosis Am J Roentgenol Radium Ther Nucl Med 124:610-624, 1975 Multiple exostoses syndrome JAAD 25:333-335, 1991 Multiple symmetric lipomatosis of the soles JAAD 26:860-862, 1992 Neurofibromatosis Ped Derm 21:506-507, 2004 Ollier syndrome – multiple enchondromas Rook p.2847, 1998, Sixth Edition Olmsted syndrome – plantar squamous cell carcinoma BJD 145:685-686, 2001 Reiter’s syndrome – keratoderma blenorrhagicum Rook p.2765-2766, 1998; Semin Arthritis Rheum 3:253-286, 1974 Sweet’s syndrome – plantar red nodules AD 134:76-79, 1998 Tuberous sclerosis – shagreen patch

Athlete’s nodules – sports-related collagenomas Cutis 50:131-135, 1992; JAAD 24:317-318, 1991 Bunion Chilblains – plantar or dorsal red papules or nodules AD 134:76-79, 1998 Delayed pressure urticaria – nodules of soles Rook p.2130, 1998, Sixth Edition; JAAD 29:954-958, 1993 Hypertrophic scar – plantar giant nodule BJD 145:1005-1007, 2001 Piezogenic nodules or papules – plantar Ped Derm 17:429-431, 2000; Hautarzt 24:114-118, 1973 Prayer nodules Clin Exp Dermatol 9:97-98, 1984 Surfer’s nodules of anterior tibial prominence, dorsum of feet, knuckles Cutis 50:131-135, 1992 Traumatic plantar urticaria – red nodules JAAD 18:144-146, 1988 Vibratory angioedema – palmoplantar red nodules

Angiosarcoma – in chronic lymphedema BJD 138:692-694, 1998 Emboli – plantar nodule Ped Derm 15:97-102, 1998 Epithelioid hemangioma – plantar nodule JAAD 49:113-116, 2003 Glomus tumor Foot Ankle Int 18:672-674, 1997 Lymphostasis verrucosa cutis

Plantar thrombotic nodules of diabetes J Dermatol 24:405-409, 1997 Polyarteritis nodosa – palmar and plantar nodules AD 130:884-889, 1994; Ped Derm 15:103-107, 1998; nodules along the course of superficial arteries around knee, anterior lower leg and dorsum of foot Ann Intern Med 89:666-676, 1978; cutaneous infarcts presenting as tender nodules Rook p.2212, 1998, Sixth Edition Pseudo-Kaposi’s sarcoma Pyogenic granuloma Spindle cell hemangioma Bolognia p.1823, 2003 Thrombosis of deep plantar vein – plantar nodule Ped Derm 15:97-102, 1998; Clin Podiatr Med Surg 13:85-89, 1996 Vascular hamartomas Vasculitis – plantar nodule JAAD 47:S263-265, 2002; Ped Derm 15:97-102, 1998 Venous malformation

Dermatomyositis – Gottron’s papules Rook p.2558-2560, 1998, Sixth Edition; Curr Opin Rheum 11:475-482, 1999 Epidermolysis bullosa acquisita Jacoud’s arthritis – non-erosive arthritis following repeated bouts of rheumatic fever or systemic lupus erythematosus Ghatan p.264, 2002, Second Edition Lupus erythematosus – systemic lupus – mimic rheumatoid nodules AD 72:49-58, 1970; rheumatoid nodules Ghatan p.264, 2002, Second Edition Rheumatoid arthritis – rheumatoid nodules Rook p.2566-2567, 1998, Sixth Edition; Eur J Radiol 27 Suppl 1:S18-24, 1998; J Rheumatol 6:286-292, 1979; palisaded neutrophilic granulomatous dermatitis of rheumatoid arthritis (rheumatoid neutrophilic dermatosis) – nodules over joints JAAD 47:251-257, 2002; AD 133:757-760, 1997; AD 125:1105-1108, 1989 Scleroderma – soft cystic nodules (focal mucinosis) over interphalangeal joints BJD 136:598-600, 1997; nodules of necrotic fibrinous material BJD 101:93-96, 1979; CREST syndrome; rheumatoid nodules Ghatan p.264, 2002, Second Edition

Bouchard’s nodes – osteoarthritis; proximal interphalangeal joints Ann Rheum Dis 48:523-527, 1953 Heberden’s nodes – distal interphalangeal joints Br Med J i:181-187, 1952

Acral dysesthesia syndrome Bleomycin-induced dermatomyositis-like rash JAAD 48:439-441, 2003 Tegafur – knuckle pad-like keratoderma Int J Dermatol 37:315-317, 1998

Foreign body granuloma Plant thorns – common blackthorn; persistent nodules of wrists and fingers Lancet i:309-310, 1960

Bejel Cryptococcosis – cryptococcal panniculitis Gonococcemia – periarticular lesions appear in crops with red macules, papules, vesicles with red halo, pustules, bullae becoming hemorrhagic and necrotic; suppurative arthritis and tenosynovitis Ann Intern Med 102:229-243, 1985 Leishmaniasis – AIDS-related visceral leishmaniasis BJD 143:1316-1318, 2000 Leprosy – digital papule JAAD 11:713-723, 1984 Lyme borreliosis – juxta-articular fibroid nodules Hautarzt 51:345-348, 2000; AD 131:1341-1342, 1995; Clin Exp Dermatol 19:394-398, 1994; BJD 128:674-678, 1993 Meningococcemia – chronic Rev Inf Dis 8:1-11, 1986 Molluscum contagiosum Mycetoma JAAD 32:311-315, 1995; Cutis 49:107-110, 1992; Australas J Dermatol 31:33-36, 1990; JAAD 6:107-111, 1982; Sabouraudia 18:91-95, 1980; AD 99:215-225, 1969 Mycobacterium haemophilum BJD 149:200-202, 2003 Mycobacterium marinum – nodule or papule of hands, elbows, knees becomes crusted ulcer or abscess; or verrucous papule; sporotrichoid; rarely widespread lesions Br Med J 300:1069-1070, 1990; AD 122:698-703, 1986; J Hyg 94:135-149, 1985 Mycobacterium tuberculosis – scrofuloderma – infected lymph node, bone, joint, lacrimal gland with overlying red-blue nodule which breaks down, ulcerates, forms fistulae, scarring with adherent fibrous masses which may be fluctuant and draining BJD 134:350-352, 1996 Nocardia – red nodule overlying wrist in patient with chronic granulomatous disease Clin Inf Dis 33:235-239, 2001 Parvovirus B19 – dermatomyositis-like Gottron’s papules Hum Pathol 31:488-497, 2000 Pinta Rheumatic fever – papules on extensor extremities near joints Rook p.2732, 1998, Sixth Edition; subcutaneous nodules around elbows or knees Rheumatol Rehab 21:195-200, 1982 Sporotrichosis Syphilis – juxta-articular nodes Ghatan p.57, Second Edition Verruca vulgaris – knuckle papules Derm Surg 27:591-593, 2001 Yaws – tertiary – juxta-articular nodes Rook p.1270, 1998, Sixth Edition

Acral persistent papular mucinosis – mimicking knuckle pads AD 140:121-126, 2004; JAAD 27:1026-1029, 1992 Amyloidosis Lichen myxedematosis – resembling acral persistent papular mucinosis BJD 144:594-596, 2001; Dermatology 185:81, 1992 Mastocytoma Caputo p.100, 2000; knuckle pads Self-healing (papular) juvenile cutaneous mucinosis – juxtaarticular nodules; also nodules of face, neck, scalp, abdomen, and thighs; arthralgias JAAD 50:S97-100, 2004; Ped Derm 20:35-39, 2003; JAAD 44:273-281, 2001; Ped Derm 14:460-462, 1997; AD 131:459-461, 1995; JAAD 11:327-332, 1984; Ann DV 107:51-57, 1980; of adult JAAD 50:121-123, 2004, BJD 143:650-651, 2000; Dermatology 192:268-270, 1996; Lyon Med 230:474-475, 1973

Bunion Extravascular necrotizing palisaded granulomas – found in systemic lupus erythematosus, Churg-Strauss disease, Wegener’s granulomatosis, lymphoproliferative disease, hepatitis, inflammatory bowel disease, Takayasu’s arteritis BJD 147:371-374, 2002 Sarcoidosis (Darier-Roussy sarcoid) JAAD 44:725-743, 2001; AD 133:882-888, 1997; Am J Med 85:731-736, 1988; nodules of DIP joints BJD 142:1052-1053, 2000 Seronegative ankylosing spondylitis – rheumatoid nodules Ghatan p.264, 2002, Second Edition

Calcinosis cutis – metastatic or dystrophic calcinosis Cutis 63:149-153, 1999; JAAD 39:527-544, 1998; tumoral calcinosis – around hip, elbow, ankle, and scapula Semin Dermatol 3:53-61, 1984; in ESRD JAAD 40:975-986, 2000 Calciphylaxis (vascular calcification cutaneous necrosis syndrome) – papules or nodules around large joints or flexures J Dermatol 28:27-31, 2001; Br J Plast Surg 53:253-255, 2000;JAAD 40:979-987, 1999; J Cutan Med Surg 2:245-248, 1998; JAAD 33:53-58, 1995; JAAD 33:954-962, 1995; JAAD 33:954-962, 1995; AD 127:225-230, 1991; tumoral calcinosis – progressively growing lobulated masses Textbook of Neonatal Dermatology, p.406, 2001 Diabetic finger pebbling (Huntley’s papules) Cutis 69:298-300, 2002 Erythropoietic protoporphyria Gout Cutis 48:445-451, 1991; Ann Rheum Dis 29:461-468, 1970 Pancreatic panniculitis – periarticular subcutaneous nodules Rook p.2414, 1998, Sixth Edition; JAAD 34:362-364, 1996; Arthritis Rheum 22:547-553, 1979 Pseudogout – pseudotophi Rook p.2653, 1998, Sixth Edition Sitosterolemia – tuberous and tendon xanthomas Ped Derm 17:447-449, 2000 Xanthomas, especially tendinous and tuberous xanthomas – cerebrotendinous xanthomatosis – mutation in sterol 27hydroxylase; increased serum cholesterol and urinary bile alcohols; normal serum cholesterol; tendon xanthomas also seen in heterozygous familial hypercholesterolemia, familial defective lipoprotein B100, familial dysbetalipoproteinemia, beta sitosterolemia JAAD 45:292-295, 2001; tuberous xanthomas Rook p.2605, 1998, Sixth Edition

Eccrine angiomatous hamartoma Ped Derm 18:117-119, 2001; Ped Derm 13:139-142, 1996; Ped Derm 14:401-402, 1997; skin-colored nodule with blue papules JAAD 41:109-111, 1999 Eccrine poroma and eccrine porocarcinoma – red nodule of ankle BJD 150:1232-1233, 2004 Fibromas Giant cell tumor of the tendon sheath – single or multiple JAAD 43:892, 2000; nodules of the fingers J Dermatol 23:290-292, 1996; J Bone Joint Surg Am 66:76-94, 1984 Keloids Lymphoma – cutaneous T-cell lymphoma Malignant melanoma of the soft parts (clear-cell sarcoma) – nodule of tendons of foot or knee Cancer 65:367-374, 1990 Myxoid cyst Neurofibroma Synovial cyst or ganglion

Necrobiotic xanthogranuloma with paraproteinemia AD 133:97-102, 1997; nodules of knees JAAD 52:729-731, 2005 Paraneoplastic scleroderma

Acanthosis nigricans Acrodermatitis chronica atrophicans – juxta-articular fibroid nodule AD 131:1341-1342, 1995 Acrokeratoelastoidosis of Costa – knuckle pads Ped Derm 19:320-322, 2002; JAAD 22:468-476, 1990; Acta DV 60:149-153, 1980; Dermatologica 107:164-168, 1953 Acrokeratosis verruciformis of Hopf Cutis laxa, acquired – fibrotic nodules over bony prominences Rook p.2020, 1998, Sixth Edition Dercum’s disease (adiposis dolorosa) – painful peri-articular lipomas and ecchymoses; lipomas feel like ‘bag of worms’ JAAD 44:132-136, 2001 Epidermolysis bullosa Epidermolytic hyperkeratosis Erythema elevatum diutinum – knuckle pads (juxta-articular nodules) JAAD 49:764-767, 2003; JAAD 28:394-398, 1994; AD 129:1043-1044, 1046-1047, 1993 Granuloma annulare – subcutaneous granuloma annulare mimicking knuckle pads JAAD 3:217-230, 1980 Hidrotic ectodermal dysplasia Id reactions Juvenile fibromatosis Knuckle pads – idiopathic (fibromatosis), keratotic knuckle pads unassociated with palmoplantar keratoderma Rook p.1555-1556, 1998, Sixth Edition; AJDC 140:915-917, 1986; trauma-induced, associated with Dupuytren’s contracture, Ledderhose’s disease, Peyronie’s disease, Bart-Pumphrey syndrome – sensorineural deafness, leukonychia, and knuckle pads; autosomal dominant Ped Derm 17:450-452, 2000; NEJM 276:202-207, 1967 Lichen nitidus – knuckle pads AD 134:1302-1303, 1998 Lichen simplex chronicus Pachydermodactyly – benign fibromatosis of fingers of young men AD 111:524, 1975 Palmoplantar keratoderma, epidermolytic (Vorner) – papules on knuckles BJD 125:496, 1991 Prurigo nodularis Psoriasis Vorner’s palmoplantar keratoderma

Bulimia nervosa – Russell’s sign (crusted knuckle nodules) Clin Orthop 343:107-109, 1997; JAAD 12:725-726, 1985; perniosis Clin Sci 61:559-567, 1981; pseudo knuckle pads (calluses on 2nd and 5th MCP joints) Psychol Med 9;429-48, 1979

Albright’s hereditary osteodystrophy – ectopic bone formation Bart-Pumphrey syndrome – knuckle pads, leukonychia, sensorineural deafness, and diffuse palmoplantar hyperkeratosis; autosomal dominant JAAD 51:292, 2004; Curr Prob Derm 14:71-116, 2002; NEJM 276:202-207, 1967

Cerebrotendinous xanthomatosis – autosomal recessive; tendon (Achilles tendon) and tuberous xanthomas Ped Derm 17:447-449, 2000 Dercum’s disease (adiposis dolorosa) – painful peri-articular lipomas JAAD 44:132-136, 2001 Distal pachydermodactyly (acquired digital fibrosis) – skin-colored nodule of the elbow JAAD 38:359-362, 1998 Ehlers-Danlos syndrome (molluscum pseudotumor) – knuckle pads Familial histiocytic dermatoarthritis – knuckle pads Familial Mediterranean fever Farber’s disease (disseminated lipogranulomatosis) – autosomal recessive; red papules and nodules of joints and tendons of hands and feet; deforming arthritis; papules, plaques, and nodules of ears, back of scalp and trunk Ped Derm 21:154-159, 2004; Rook p.2642, 1998, Sixth Edition; Am J Dis Child 84:449-500, 1952 Fibroblastic rheumatism – symmetrical polyarthritis, nodules over joints and on palms, elbows, knees, ears, neck, Raynaud’s phenomenon, sclerodactyly; skin lesions resolve spontaneously AD 139:657-662, 2003; AD 131:710-712, 1995; Clin Exp Dermatol 19:268-270, 1994; Rev Rheum Ed Fr 47:345-351, 1980 François syndrome (dermochondrocorneal dystrophy) – knuckle pads; nodules on hands, nose, and ears Ann DV 104:475-478, 1977; AD 124:424-428, 1988 Hunter syndrome – MPS II Ped Derm 12:370-372, 1995 Infantile digital fibromatosis Infantile systemic hyalinosis – autosomal recessive; juxtaarticular nodules (knuckle pads), synophrys, thickened skin, perianal nodules, dusky red plaques of buttocks, gingival hypertrophy, joint contractures, osteopenia, growth failure, diarrhea, frequent infections, facial red papules JAAD 50:S61-64, 2004 Juvenile hyaline fibromatosis – pearly white papules of face and neck; larger papules and nodules around nose, behind ears, on fingertips, knuckle pads; multiple subcutaneous nodules of scalp, trunk, and extremities, papillomatous perianal papules; joint contractures, skeletal lesions, gingival hyperplasia, stunted growth Textbook of Neonatal Dermatology, p.444-445, 2001; Caputo p.54, 2000; AD 121:1062-1063, 1985; AD 107:574-579, 1973 Knuckle pads, leukonychia, and deafness syndrome Ghatan p.159, 2002, Second Edition Knuckle pads with palmoplantar keratoderma and acrokeratoelastoidosis Ledderhose’s nodules (plantar fibromatosis) JAAD 41:106-108, 1999; Dupuytren’s contracture (palmar fibromatosis) and/or Peyronie’s disease – knuckle pads Lipoid proteinosis – xanthoma-like nodules of elbows; nodules of finger joints, knees BJD 151:413-423, 2004; JID 120:345-350, 2003; BJD 148:180-182, 2003; Hum Molec Genet 11:833-840, 2002; Rook p.2641, 1998, Sixth Edition; Acta Paediatr 85:1003-1005, 1996; JAAD 27:293-297, 1992 Mal de Meleda (recessive transgressive palmoplantar keratoderma) – knuckle pads Curr Prob Derm 14:71-116, 2002; Ped Derm 14:186-191, 1997 Multicentric reticulohistiocytosis AD 140:919-921, 2004; Rook p.2325-2326, 1998, Sixth Edition; BJD 133:71-76, 1995; AD 126:251-252, 1990; Clin Exp Dermatol 15:1-6, 1990; Oral Surg Oral Med Oral Pathol 65:721-725, 1988; Pathology 17:601-608, 1985; JAAD 11:713-723, 1984; AD 97:543-547, 1968; mimicking dermatomyositis JAAD 48:S11-14, 2003 Neurofibromatosis

Pachydermoperiostosis – knuckle pads J Dermatol 27:106-109, 2000 Palmoplantar keratoderma, Vorner – knuckle pads Polyfibromatosis syndrome – Dupuytren’s contracture, knuckle pads, Peyronie’s disease, keloids, or plantar fibromatosis Rook p.2044, 1998, Sixth Edition; stimulation by phenytoin BJD 100:335-341, 1979 Pseudohypoparathyroidism – periarticular calcified nodules JAAD 15:353-356, 1986 Reflex sympathetic dystrophy with chilblain-like lesions – digital papule Rowell’s syndrome – lupus erythematosus and erythema multiforme-like syndrome – perniotic lesions JAAD 21:374-377, 1989 Stiff skin syndrome – knuckle pads Ped Derm 3:48-53, 1985 Vohwinkel’s syndrome – knuckle papules, palmoplantar keratoderma, ichthyosis, pseudoainhum JAAD 44:376-378, 2001

Callosities, occupational (carpenters, live chicken hangers Contact Derm 17:13-16, 1987), frictional, bulemic Chilblains – tender, pruritic red or purple digital papules JAAD 45:924-929, 2001; Rook p.960-961, 1998, Sixth Edition Garrod’s pads – violinist’s knuckles (2nd and 3rd knuckles) – thickened skin over the interphalangeal joints from intense flexion of tendons of fingers Cutis 62:261-262, 1998 Piezogenic wrist papules Scars – mimic knuckle pads Skier’s thumb Acta Orthop Belg 65:440-446, 1999 Surfer’s nodules of anterior tibial prominence, dorsum of feet, knuckles Cutis 50:131-135, 1992

Churg-Strauss syndrome – elbow papules and nodules JAAD 37:199-203, 1997; umbilicated nodules of elbows BJD 150:598-600, 2004 Vasculitis

NODULES, KNEE Acral dysesthesia syndrome Bursitis Callosities Connective tissue nevi Ehlers-Danlos syndrome (molluscoid tumors) Elastoma Elastosis perforans serpiginosa Enchondromas Epidermolysis bullosa acquisita Epidermolysis bullosa dystrophica Erythema elevatum diutinum AD 129:1043-1044, 1046-1047, 1993 Fibroblastic rheumatism – papulonodules on elbows and knees Foreign body granuloma Ganglion cyst Gout Cutis 48:445-451, 1991; Ann Rheum Dis 29:461-468, 1970 Granuloma annulare

IgM storage papule – pink or skin-colored Hypertrophic scar Juvenile hyaline fibromatosis AD 112:86-88, 1976 Keloids Keratoacanthoma Leprosy Lobomycosis Malignant melanoma of the soft parts (clear-cell sarcoma) – nodule of knee Cancer 65:367-374, 1990 Multicentric reticulohistiocytosis Rook p.2325-2326, 1998, Sixth Edition; BJD 133:71-76, 1995; AD 126:251-252, 1990; Clin Exp Dermatol 15:1-6, 1990; Oral Surg Oral Med Oral Pathol 65:721-725, 1988; Pathology 17:601-608, 1985; JAAD 11:713-723, 1984; JAAD 11:713-723, 1984; AD 97:543-547, 1968 Mycobacterium marinum – nodule or papule of hands, elbows, knees becomes crusted ulcer or abscess; or verrucous papule; sporotrichoid; rarely widespread lesions Br Med J 300:1069-1070, 1990; AD 122:698-703, 1986; J Hyg 94:135-149, 1985 Nevus lipomatosis superficialis Papular urticaria Prayer nodules – on knees and ankles of Shi’ite Muslims Cutis 38:281-286, 1986 Prurigo nodularis Rheumatoid nodule Sarcoid Surfer’s nodules JAMA 201:134-136, 1967 Vascular hamartomas Xanthomas – tuberous xanthomas Rook p.2605, 1998, Sixth Edition

Dermatomyositis with cutaneous mucinosis AD 126:1639-1644, 1990 Lupus erythematosus – nodular episcleritis Morphea – keloidal (Addisonian keloid) Int J Dermatol 31:422-423, 1992; generalized morphea – keloidal nodules Rook p.2511, 1998, Sixth Edition Rheumatoid arthritis – rheumatoid nodules J Rheumatol 6:286-292, 1979; Rook p.2566-2567, 1998, Sixth Edition; Eur J Radiol 27 Suppl 1:S18-24, 1998

Accessory tragus Congenital cutaneous plate-like osteoma cutis – mountain range-like topography Ped Derm 10:371-376, 1993 Congenital lipoblastomatosis Ped Derm 15:210-213, 1998 Congenital papillated aprocrine cystadenoma JAAD 11:374-376, 1984 Torus mandibularis Cutis 71:363-364, 2003

Iododerma JAAD 36:1014-1016, 1997

Cutaneous geode – nodular crateriform scar JAAD 14:1085-1086, 1986

Actinomycosis Adiaspiromycosis – cutaneous adiaspiromycosis (Chrysosporium species) – hyperpigmented plaque with white-yellow papules, ulcerated nodules, hyperkeratotic nodules, crusted nodules, multilobulated nodules JAAD S113-117, 2004 African histoplasmosis Alternariosis Bacillary angiomatosis SKINmed 4:215, 2005 Candida albicans – in immunosuppressed host Carbuncle Chromomycosis BJD 146:704, 2002; AD 133:1027-1032, 1997; BJD 96:454-458, 1977; AD 104:476-485, 1971 Coccidioidomycosis Condyloma acuminata Tyring p.263, 2002; Rook p.3171, 1998, Sixth Edition Furuncle Keloidal blastomycosis (lobomycosis) – Loboa loboi; legs, arms, face Rook p.1359-1360, 1998, Sixth Edition; JAAD 29:134-136, 139-140, 1993; Cutis 46:227-234, 1990 Leishmaniasis – leishmaniasis recidivans (lupoid leishmaniasis) – brown-red or brown-yellow papules close to scar of previously healed lesion; resemble lupus vulgaris; may ulcerate or form concentric rings; keloidal form, verrucous form of legs, extensive psoriasiform dermatitis; diffuse cutaneous (Leishmania aethiopica, L. mexicana) Rook p.1414, 1998, Sixth Edition; JAAD 23:368-371, 1990 Leprosy Lyme disease – lymphocytoma cutis, Borrelia-associated JAAD 23:401-410, 1990 Milker’s nodule Molluscum contagiosum Tyring p.64, 2002; Cutis 60:29-34, 1997 Mycetoma Mycobacterium tuberculosis – multilobulated tumor of the earlobe BJD 150:370-371, 2004 Rhinosporidiosis – vascular nodules Rook p.1360, 1998, Sixth Edition; Arch Otolaryngol 102:308-312, 1976 Subcutaneous phaeohyphomycosis Syphilis – granulomatous, tertiary AD 125:551-556, 1989 Yaws Int J Derm 21:220-3, 1982

Amyloidosis – nodular (tumefactive) amyloidosis; of great toe JAAD 49:307-310, 2003 Langerhans cell histiocytosis Textbook of Neonatal Dermatology, p.438, 2001 Mastocytosis Diagnostic Challenges Vol V25-27, 1994; AD 127:405-410, 1991 Pretibial mucin AD 129:1152-1156, 1993 Pretibial myxedema Verruciform xanthoma of toes in patient with Milroy’s disease due to persistent leg edema Ped Derm 20:44-47, 2003; JAAD 20:313-317, 1989 Xanthoma disseminatum – mountain range-like topography AD 121:1313-1317, 1985

Crohn’s disease, metastatic Ped Derm 13:25-28, 1996 Dissecting cellulitis of the scalp Hidradenitis suppurativa Malacoplakia JAAD 23:947-948, 1990 Rosai-Dorfman disease (sinus histiocytosis with lymphadenopathy) AD 114:191-197, 1978 Sarcoid – keloid-like lesions Rook p.2694, 1998, Sixth Edition

Calcinosis cutis, dystrophic Cutis 60:259-262, 1997; calcinosis universalis in dermatomyositis NEJM 349:1246, 1974, 2003; idiopathic calcinosis of the scrotum; tumoral calcinosis – progressively growing lobulated masses Textbook of Neonatal Dermatology, p.406, 2001 Cushing’s syndrome – buffalo hump Endometriosis – cutaneous deciduosis JAAD 43:102-107, 2000 Goiter Gout Cutis 48:445-451, 1991; Ann Rheum Dis 29:461-468, 1970 Xanthomas – tuberous xanthomas JAAD 19:95-111, 1988; Cutis 59:315-317, 1997

Acrochordon, giant Apocrine cystadenoma – congenital papillated apocrine cystadenoma JAAD 11:374-376, 1984 Apocrine gland carcinoma – axillary mass Am J Med 115:677-679, 2003 Atypical fibroxanthoma AD 135:1113-1118, 1999; JAAD 35:262-264, 1996 Basal cell carcinoma JAAD 52:149-151, 2005; Cutis 58:289-292, 1996; Neuroradiology 38:575-577, 1996; BJD 127:164-167, 1992; J Derm Surg Oncol 12:459-464, 1986 Cervical cysts Chordoma AD 133:179-1584, 1997 Clear cell acanthoma JAAD 44:314-316, 2001 Clear cell eccrine porocarcinoma BJD 149:1059-1063, 2003 Clear cell hidradenoma (eccrine acrospiroma) – head, neck, upper extremities Ped Derm 18:356-358, 2001; giant eccrine acrospiroma JAAD 23:663-668, 1990 Clear cell hidradenocarcinoma JAAD 12:15-20, 1985 Cylindrocarcinoma BJD 145:653-656, 2001 Cylindroma JAAD 33:199-206, 1995; BJD 145:653-656, 2001; JAAD 19:397-400, 1988 Dermatofibroma – multinodular hemosiderotic dermatofibroma Dermatologica 181:320-323, 1990 Dermatofibrosarcoma protuberans Sem Cut Med Surg 21:159-165, 2002; JAAD 35:355-374, 1996 Eccrine gland carcinoma JAAD 20:693-696, 1989 Eccrine porocarcinoma – multilobulated or cauliflower-like nodule BJD 152:1051-1055, 2005; JAAD 49:S252-254, 2003; J Derm Surg 25:733-735, 1999; JAAD 35:860-864, 1996 Eccrine poroma JAAD 53:539-541, 2005 Epidermal nevus Epidermoid cyst – ruptured with scarring; multiloculated epidermoid cyst BJD 151:943-945, 2004 Giant cell tumor of the tendon sheath – multilobulated single or multiple JAAD 43:892, 2000; nodules of the fingers J Dermatol 23:290-292, 1996; J Bone Joint Surg Am 66:76-94, 1984

Giant folliculosebaceous cystic hamartoma – multinodular plaque of scalp AD 141:1035-1040, 2005 Infantile digital fibromatosis Intradermal nevus, lobulated JAAD 24:74-77, 1991 Kaposi’s sarcoma (KS) AD 141:1311-1316, 2005; BJD 145:847-849, 2001; Rook p.1063,2358-2360, 1998, Sixth Edition; JAAD 38:143-175, 1998; Dermatology 190:324-326, 1995; keloidal Kaposi’s sarcoma Dermatology (Basel) 189:271-274, 1994; lymphangioma-like variant of KS AD 139:381-386, 2003 Keloids Rook p.2056-2057, 1998, Sixth Edition; keloids in Ehlers-Danlos syndrome, progeria, Rubinstein-Taybi syndrome Ped Derm 12:387-389, 1995; plantar keloid JAAD 48:131-134, 2003 Keratoacanthoma JAAD 19:826-830, 1988 Leiomyosarcoma J D Surg Oncol 9:283-287, 1983 Leukemia cutis, including congenital leukemia cutis JAAD 34:375-378, 1996 Lipoblastomatosis Textbook of Neonatal Dermatology, p.429, 2001 Lipomas, multiple Lymphocytoma cutis Lymphoma – cutaneous T-cell lymphoma Rook p.2377, 1998, Sixth Edition; JAAD 25:345-349, 1991; HTLV-1 (keloid-like) JAAD 34:69-76, 1996; pilotropic (follicular) CTCL AD 138:191-198, 2002; gamma/delta T-cell lymphoma AD 136:1024-1032, 2000; CD30 + lymphoma of AIDS Tyring p.375, 2002; B-cell lymphoma JAAD 29:359-362, 1993; JAAD 16:518-526, 1987; large B-cell lymphoma BJD 149:542-553, 2003; primary cutaneous large B-cell lymphoma of the legs AD 132:1304-1308, 1996; reticulohistiocytoma of the dorsum (B-cell lymphoma) JAAD 18:259-272, 1988 Malignant blue nevus Rook p.1752, 1998, Sixth Edition; JAAD 19:712-722, 1988 Malignant fibrous histiocytoma Malignant histiocytosis X Cancer 54:347-352, 1984 Malignant proliferating trichilemmal tumor BJD 150:156-157, 2004 Melanocytic nevus Rook p.1722-1723, 1998, Sixth Edition; giant congenital melanocytic nevi with proliferative nodules AD 140:83-88, 2004 Melanoma Curr Prob Derm 14:41-70, 2002; Cutis 69:353-356, 2002; Semin Oncol 2:5-118, 1975; polypoid melanoma JAAD 23:880-884, 1990 Melanoma of the soft parts (clear cell sarcoma) JAAD 38:815-819, 1998 Merkel cell carcinoma Metastases – carcinoma telangiectoides; lung cancer with emboli in pulmonary venous circulation; nodules of scalp resembling cylindromas Rook p.2371, 1998, Sixth Edition; Cancer 19:162-168, 1966; cutaneous metastases (adenocarcinoma) – lymphangioma-like lesions JAAD 30:1031-1032, 1994; metastatic prostate carcinoma mimicking cylindromas JAAD 33:161-182, 1995; rhinophyma-like metastatic carcinoma Cutis 57:33-36, 1996; breast carcinoma Mucinous carcinoma of skin JAAD 36:323-326, 1997 Mucinous nevus (connective tissue hamartoma) AD 141:897-902, 2005 Neural fibrolipoma AD 135:707-712, 1999 Neuroectodermal tumors – multiple primitive neuroectodermal tumors JAAD 31:356-361, 1994 Neurofibroma – diffuse neurofibroma JAAD 48:938-940, 2003 Neurogenic sarcoma

Nevus lipomatosis superficialis – mountain range-like lesions Cutis 72:237-238, 2003; AD 128:1395-1400, 1992 Nevus sebaceus Pilomatrixoma Plexiform schwannoma of the foot Eur Radiol 9:1653-1655, 1999 Polymorphous sweat gland carcinoma JAAD 46:914-916, 2002 Proliferating pilar cyst Cutis 48:49-52, 1991 Regressing atypical histiocytosis AD 126:1609-1616, 1990 Rhabdomyosarcoma Textbook of Neonatal Dermatology, p.441, 2001 Salivary pleomorphic adenoma Cutis 63:167-168, 1999 Sebaceous carcinoma JAAD 47:950-953, 2002 Seborrheic keratosis Spindle cell tumor Squamous cell carcinoma – cauliflower-like tumor Caputo p.77, 2000; complicating venous stasis ulcers South Med J 58:779-781, 1965 Syringocystadenoma papilliferum Rook p.1704, 1998, Sixth Edition Syringomatous carcinoma – multilobulated digital nodule BJD 144:438-439, 2001 Torus palatinus

Necrobiotic xanthogranuloma with paraproteinemia JAAD 52:729-731, 2005

Acne rosacea – rhinophyma Rook p.2104-2110, 1998, Sixth Edition Cutis verticis gyrata – acromegaly, Apert’s syndrome, amyloidosis, leukemia, myxedema, syphilis, pachydermoperiostosis, tuberous sclerosis Ghatan p.128, 2002, Second Edition Cystic acne – exophytic abscesses of chin in follicular occlusion triad JAAD 48:S47-50, 2003 Erythema elevatum diutinum Tyring p.358, 2002; BJD 143:415-420, 2000; keloid-like lesions of erythema elevatum diutinum in AIDS JAAD 28:919-922, 1993 Follicular mucinosis JAAD 20:441-446, 1989 Granuloma annulare, including subcutaneous granuloma annulare Myospherulosis (subcutaneous spherulocystic disease) – multilobulated abdominal nodule AD 138:1309-1314, 2002 Periumbilical pseudoxanthoma elasticum JAAD 39:338-344, 1998 Pseudofolliculitis barbae with keloids Rhinophyma Rook p.3581, 1998, Sixth Edition

Benign symmetrical lipomatosis AD 126:235-240, 1990; JAAD 18:359-362, 1988 Blue rubber bleb nevus syndrome Carney complex – cutaneous myxoma AD 141:916-918, 2005 Congenital diffuse lipomatosis Delleman-Oorthuys syndrome Clin Genet 25:470-472, 1984

Dercum’s disease (adiposis dolorosa) – painful peri-articular lipomas and ecchymoses; lipomas feel like ‘bag of worms’ JAAD 44:132-136, 2001 Goeminne syndrome – X-linked; torticollis, keloids, cryptorchidism, renal dysplasia AD 137:1429-1434, 2001; Acta Genet Med (Roma) 17:439-467, 1968 Goltz’s syndrome – raspberry-like papillomas of lips, perineum, ears, fingers, toes, buccal mucosa, and esophagus Multiple lipomas due to intracranial lesions (Frohlich syndrome) Lipomatosis of Touraine and Renault Familial multiple lipomatosis JAAD 15:275-279, 1986 Infantile myofibromatosis Maffucci’s syndrome Rook p.2295, 1998, Sixth Edition; Syndromes of the Head and Neck, 3rd Edition, pp.384, 1990 Michelin tire baby syndrome – smooth muscle hamartomas JAAD 28:364-370, 1993; nevus lipomatosus superficialis AD 115:978-979, 1979; AD 100:320-323, 1969 Multicentric reticulohistiocytosis – digital papule; knuckle pads yellow papules and plaques JAAD 44:373-375, 2001; Rook p.2325-2326, 1998, Sixth Edition; AD 126:251-252, 1990; Oral Surg Oral Med Oral Pathol 65:721-725, 1988; Pathology 17:601-608, 1985; JAAD 11:713-723, 1984; AD 97:543-547, 1968 Multiple symmetrical lipomatosis Muscular dystrophy – pseudo-athletic appearance Neurofibromatosis type I Neurolipomatosis Alsberg Nodular lipomatosis of Krabbe and Bartels Noonan’s syndrome – webbed neck, short stature, malformed ears, nevi, keloids, transient lymphedema, ulerythema ophyrogenes, keratosis follicularis spinulosa decalvans JAAD 46:161-183, 2002; Rook p.3016, 1998, Sixth Edition; J Med Genet 24:9-13, 1987 Proteus syndrome – multiple lipomas, connective tissue nevi Ped Derm 11:222-226, 1994 Pseudolipomatosis of Verneuil and Patain Rubinstein-Taybi syndrome – keloids; CREB-binding protein (transcriptional coactivator) AD 137:1429-1434, 2001 Steatocystoma multiplex Tuberous sclerosis – adenoma sebaceum JAAD 20:918-920, 1989; non-symmetrical subcutaneous lipomatosis; Koenen’s tumors Caputo p.49, 2000 Turner’s syndrome – keloids JAAD 46:161-183, 2002; West J Med 137:32-44, 1982

Verrucous hyperplasia of the stump

Angiolymphoid hyperplasia with eosinophilia AD 136:837-839, 2000; JAAD 37:887-920, 1997; Cutis 60:281-282, 1997 Elephantiasis nostras of penis AD 137:1095-1100, 2001 Glomus tumors – multiple or plaque type; hemi-facial JAAD 45:239-245, 2001; Ped Derm 18:223-226, 2001; AD 127:1717-1722, 1991 Hemangioma Hemolymphangioma Klippel-Trenaunay-Weber syndrome – aneurysmal venous dilatation JAAD 18:1169-1172, 1988 Lipomyxangioma

Lymphangiectasia (acquired lymphangioma) – due to scarring processes such as recurrent infections, radiotherapy, scrofuloderma, scleroderma, keloids, tumors, tuberculosis, repeated trauma BJD 132:1014-1016, 1996 Lymphangioma circumscriptum – frog spawn-like appearance BJD 83:519-527, 1970 Lymphangiosarcoma (Stewart-Treves tumor) – red-brown or ecchymotic patch, nodules, plaques in lymphedematous limb Cancer 1:64-81, 1948 Lymphedema of vulva – chronic infection, recurrent streptococcal cellulitis Genital Skin Disorders, Fischer and Margesson, CV Mosby p.222-223, 1998 Lymphostasis verrucosa cutis Port wine stain with epithelial and mesenchymal hamartomas JAAD 50:608-612, 2004 Pyogenic granuloma Venous malformations AD 139:1409-1416, 2003

Dermatomyositis – calcinosis cutis AD 140:365-366, 2004; juvenile dermatomyositis with calcinosis cutis BJD 144:894-897, 2001 Lupus erythematosus – nodular mucinosis of SLE Cutis 72:366-371, 2003; BJD 137:450-453, 1997; J Rheumatol 21:940-941, 1994; JAAD 27:312-315, 1992; Int J Derm 31:649-652, 1992 Rheumatoid arthritis – rheumatoid nodules JAAD 46:161-183, 2002; Rook p.2566, 1998, Sixth Edition; rheumatoid nodulosis Arthritis Rheum 29:1278-1283, 1986; J Rheumatol 6:286-292, 1979; rheumatoid vasculitis BJD 147:905-913, 2002 Still’s disease (juvenile rheumatoid nodule) Ann Rheum Dis 17:278-283, 1958

Congenital cartilaginous rests of the neck Cutis 58:293-294, 1996

Indinivir – multiple angiolipomas due to protease inhibitors JAAD 42:129-131, 2000; BJD 143:1113-1114, 2000 Methotrexate-induced accelerated rheumatoid nodulosis Medicine 80:271-278, 2001; J Dermatol 26:46-464, 1999; JAAD 39:359-362, 1998 Minocycline-induced p-ANCA + cutaneous polyarteritis nodosa – multiple subcutaneous nodules of the legs Eur J Dermatol 13:366-368, 2003; JAAD 44:198-206, 2001 Zidovudine – multiple subcutaneous nodules due to insect bites JAAD 46:284-293, 2002

Aluminum-containing allergen extracts Eur J Dermatol 11:138-140, 2001 Oleomas – multiple subcutaneous oleomas due to injection with sesame seed oil BJD 149:1289-1290, 2003; JAAD 42:292-294, 2000 Polyvinyl pyrrolidone injections

Acanthamoeba J Clin Inf Dis 20:1207-1216, 1995 Actinomycosis (Actinomyces israelii) – primary cutaneous – subcutaneous nodules with draining sinuses Hum Pathol 4:319-330, 1973 African histoplasmosis Alveolar echinococcosis JAAD 34:873-877, 1996 Bacillary angiomatosis BJD 126:535-541, 1992 Cat scratch disease AIDS 3:751-753, 1989 Chromomycosis Mycoses 31:343-352, 1988 Cysticercosis JAAD 50:S14-17, 2004; Int J Dermatol 34:574-579, 1995 Fusarium solanae, disseminated JAAD 47:659-666, 2002; J Med Vet Mycol 24:105-111, 1986 Leishmania JAAD 36:847-849, 1997; Int J Dermatol 26:300-304, 1987; diffuse cutaneous leishmaniasis Leprosy – reactional state in tuberculoid leprosy Indian J Lepr 65:239-242, 1993 Loiasis Clin Infect Dis 29:680-682, 1999 Lymphogranuloma venereum – sign of the groove Mycetoma Mycobacterium avium-intracellulare BJD 142:789-793, 2000; J Dermatol 25:384-390, 1998; BJD 130:785-790, 1994 Mycobacterium chelonae AD 123:1603-1604, 1987; M. kansasii, M. haemophilum BJD 131:379, 1994; Rook p.1213, 1998, Sixth Edition; M. scrofulaceum, M. marinum, M. fortuitum, M. abscessus, M. avium-intracellulare Clin Inf Dis 19:263-273, 1994 Nocardia – abscesses J Dermatol 26:829-833, 1999; lymphocutaneous nocardiosis Acta DV 74:447-448, 1994 Onchocerciasis – onchocercomas AD 140:1161-1166, 2004; Rook p.1383, 1998, Sixth Edition; BJD 121:187-198, 1989 Pseudomonas aeruginosa Cutis 63:161-163, 1999 Rheumatic fever JAAD 8:724-728, 1983 Rhinosporidiosis Indian J Pathol Microbiol 40:95-98, 1997 Streptocerciasis – Mansonella streptocerca – similar rash to onchocerciasis; acute or lichenified papules with widespread lichenification and hypopigmented macules Rook p.1384, 1998, Sixth Edition Syphilis – subcutaneous secondary syphilis Toxocariasis – (Toxocara canis, T. cati, T. leonensis) visceral larva migrans – migrating panniculitis Dermatologica 144:129-143, 1972 Yaws – tertiary – gumma; multiple subcutaneous nodules; overlying skin ulcerates with purulent discharge; atrophic pigmented scars Rook p.1271, 1998, Sixth Edition Zygomycosis – subcutaneous zygomycosis JAAD 30:904-908, 1994

Amyloidosis – subcutaneous nodular amyloidosis Hum Pathol 32:346-348, 2001; β2-microglobulin amyloidosis – shoulder pain, carpal tunnel syndrome, flexor tendon deposits of hands, lichenoid papules, hyperpigmentation, subcutaneous nodules (amyloidomas) Int J Exp Clin Inves 4:187-211, 1997; South Med J 88:876-878, 1995; Arch Pathol Lab Med 118:651-653, 1994; J Clin Pathol 46:771-772, 1993; Nephron 55:312-315,

1990; Nephron 53:73-75, 1989; dialysis-related β2-microglobulin amyloidosis of buttocks BJD 149:400-404, 2003; bilateral popliteal tumors Am J Kidney dis 12:323-325, 1988 Langerhans cell histiocytosis Clin Exp Dermatol 27:135-137, 2002 Self-healing juvenile cutaneous mucinosis JAAD 50:S97-100, 2004; Lyon Med 230:474-475, 1973

Acute rheumatic fever Indian Heart J 45:463-467, 1993 Fat necrosis – nodular cystic fat necrosis JAAD 21:493-498, 1989; membranous fat necrosis AD 129:1331, 1334, 1993 Hidradenitis suppurativa Derm Surg 26:638-643, 2000; BJD 141:231-239, 1999; Rook p.1176-1179, 1998, Sixth Edition Lymphadenopathy, reactive Proliferative myositis Clin Exp Dermatol 22:101-103, 1997 Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) Int J Derm 37:271-274, 1998 Sarcoidosis Clin Exp Dermatol 19:356-358, 1994 Subcutaneous fat necrosis of the newborn – red to bluish-red firm nodules and/or plaques; buttocks, thighs, shoulders, back, cheeks, and arms Clin Pediatr 20:748-750, 1981

Cerebrotendinous xanthomatosis – mutation of sterol 27-hydroxylase (mitochondrial enzyme); increased cholestanol BJD 142:378-380, 2000 Dermal hematopoiesis, neonatal Ped Derm 14:383-386, 1997 Gout AD 134:499-504, 1998; Arthritis Care Res 9:74-77, 1996 Nephrogenic fibrosing dermopathy JAAD 48:42-47, 2003 Oxalate granulomas BJD 128:690-692, 1993 Pancreatic fat necrosis Am J Med Sci 319:68-72, 2000 Pretibial myxedema Tendinous xanthomas

Cerebrotendinous xanthomatosis – periarticular tendon xanthomas; mutation in sterol 27-hydroxylase; increased serum cholestanol and urinary bile alcohols; normal serum cholesterol JAAD 45:292-295, 2001 Phytosterolemia (beta sitosterolemia) Familial hypercholesterolemia Familial combined hyperlipidemia Familial type III hyperlipoproteinemia

Weber-Christian disease or recurrent nodular panniculitis Rev Clin Esp 196:405-406, 410, 1996 (Sp)

Angiolipoma – arms, legs, abdomen AD 126:666-667, 669, 1990; AD 82:924-931, 1960 Carcinoid tumors Tumori 76:44-47, 1990 Dermatofibroma – metastasizing cellular dermatofibroma Am J Surg Pathol 20:1361-1367, 1996 Elastofibromas JAAD 50:126-129, 2004 Folliculosebaceous cystic hamartomas AD 139:803-808, 2003 Granular cell tumors AD 140:353-358, 2004; AD 140:353-358, 2004; JAAD 47:S180-182, 2002; BJD 143:906-907, 2000; JAAD 36:327-330, 1997; AD 126:1051-1056, 1990 Infantile myofibromatosis – single or multiple (hundreds); head, neck, trunk Ped Derm 18:305-307, 2001; JAAD 41:508, 1999; AD 134:625-630, 1998; Cancer 48:1807-1818, 1981

Keratoacanthomas of Ferguson-Smith Leiomyomas Leiomyosarcomas J Exp Clin Cancer Res 17:405-407, 1998; Am J Pediatr Hematol Oncol 14:265-268, 1992 Leukemia – leukemia cutis (AML,AMML) AD 134:1477-1482, 1998; chronic lymphocytic leukemia – multiple lymph nodes Lipoblastomas, including congenital lipoblastomatosis Ped Derm 15:210-213, 1998 Lipomatosis Rook p.2431, 1998, Sixth Edition; Br J Clin Pract 28:101-102, 1974; of shoulder girdle Ann Intern Med 117:749-752, 1992; spindle cell lipomas JAAD 48:82-85, 2003 Lymphoma – intravascular B-cell lymphoma mimicking erythema nodosum J Cutan Pathol 27:413-418, 2000; subcutaneous panniculitis-like T-cell lymphoma JAAD 50:465-459, 2004; cytophagic histiocytic panniculitis and subcutaneous panniculitis-like T-cell lymphoma JAAD 50:S18-22, 2004; AD 136:889-896, 2000; gamma/delta T-cell lymphoma AD 136:1024-1032, 2000; lymphadenopathy Metastases – multiple primary sites; squamous cell carcinoma of the cervix Int J Gynecol Cancer 11:78-80, 2001; metastatic carcinoid AD 141:93-98, 2005; lymphadenopathy Neurilemmomatosis BJD 125:466-468, 1991 Plantar fibromatosis Cutis 68:219-222, 2001 Post-transplantation lymphoproliferative disorder AD 140:1140-1164, 2004 Progressive nodular fibrosis of the skin Ann DV 104:141-146, 1977 Schwannomatosis BJD 148:804-809, 2003

Granuloma annulare, subcutaneous (‘pseudorheumatoid nodule’) Pediatr 100:965-967, 1997 Painful piezogenic pedal papules Caputo p.177, 2000; JAAD 36:780-781, 1997

Autoimmune lymphoproliferative syndrome NEJM 351:1409-1418, 2004 Bannayan-Riley-Ruvalcaba-Zonana syndrome (PTEN phosphatase and tensin homolog hamartoma) – dolicocephaly, frontal bossing, macrocephaly, ocular hypertelorism, long philtrum, thin upper lip, broad mouth, relative micrognathia, lipomas, penile or vulvar lentigines, facial verruca-like or acanthosis nigricans-like papules, multiple acrochordons, angiokeratomas, transverse palmar crease, accessory nipple, syndactyly, brachydactyly, vascular malformations, arteriovenous malformations, lymphangiokeratoma, goiter, hamartomatous intestinal polyposis JAAD 53:639-643, 2005 Benign symmetrical lipomatosis (horse-collar neck) (Madelung’s deformity) (multiple symmetric lipomatosis) – autosomal dominant; male alcoholics; lipomas of head, neck, shoulder girdle, proximal extremities; neuropathy BJD 143:684-686, 2000; Rook p.2433-2434, 1998, Sixth Edition; Skeletel Radiol 24:72-73, 1995; of the lower abdomen, thighs Int J Dermatol 32:594-597, 1993; of the soles JAAD 26:860-862, 1992 Birt-Hogg-Dube syndrome – giant disfiguring lipomas JAAD 50:810-812, 2004 Congenital generalized myofibromatosis – autosomal recessive or dominant Ped Derm 21:154-159, 2004; Pediatr Pathol Lab Med 15:571-587, 1995 Congenital self-healing reticulohistiocytosis (Hashimoto-Pritzker disease) – multiple congenital purple nodules

Dercum’s disease (adiposis dolorosa) – painful peri-articular lipomas JAAD 44:132-136, 2001 Ekbom’s syndrome (myoclonic epilepsy and ragged muscle fibers) (mitochondrial syndrome) – cervical lipomas JAAD 39:819-823, 1998 Encephalocraniocutaneous lipomatosis – unilateral or bilateral skin-colored or yellow domed papules or nodules of scalp (hairless plaque), head, and neck; ipsilateral cranial and facial asymmetry, cranial and ocular abnormalities, spasticity, mental retardation Ped Derm 10:164-168, 1993; Arch Neurol 22:144-155, 1970 Familial histiocytic dermatoarthritis syndrome – uveitis, destructive arthritis; papulonodular eruption Am J Med 54:793-800, 1973 Familial multiple lipomatosis; familial angiolipomatosis Arch Pathol Lab Med 123:946-948, 1999 Farber’s disease (lipogranulomatosis) – deformed or stiff joints and periarticular subcutaneous nodules Eur J Ped 157:515-516, 1998; AD 130:1350-1354, 1994 Fibrodysplasia ossificans progressiva Gardner’s syndrome – multiple epidermoid cysts, osteomas (especially of facial bones), lipomas, fibromas Am J Surg 143:405-408, 1982; Clin Exp Dermatol 1:75-82, 1976 Hemihyperplasia-multiple lipomatosis (HML) syndrome – extensive congenital vascular stain, compressible blue nodule, multiple subcutaneous nodules, hemihypertrophy, syndactyly, thickened but not cerebriform soles, dermatomyofibroma Soc Ped Derm Annual Meeting, July 2005; Am J Med Genet 130A:111-122, 2004; Am J Med Genet 79:311-318, 1998 Infantile myofibromatosis J Dermatol 28:379-382, 2001; AD 136:597-600, 2000; JAAD 41:508, 1999 Juvenile hyaline fibromatosis – large subcutaneous nodules of scalp, trunk and extremities Ped Derm 18:400-402, 2001; Pathol Int 48:230-236, 1998 Leri-Weill dyschondrosteosis – mesomelic short stature syndrome with Madelung’s deformity; SHOX haploinsufficiency like Turner’s syndrome JAAD 50:767-776, 2004 Maffucci’s syndrome – enchondromas, angiomas, cartilaginous nodules Cutis 69:21-22, 2002; Rook p.2847, 1998, Sixth Edition; Dermatologic Clinics 13:73-78, 1995; JAAD 29:894-899, 1993 Neurofibromatosis type I Dermatol Clinics 13:105-111, 1995; Curr Prob Cancer 7:1-34, 1982; NEJM 305:1617-1627, 1981 Penchaszadeh syndrome (nasopalpebral lipoma-coloboma syndrome) – eyelid lipoma Am J Med Genet 11:397-410, 1982 Polyneuropathy with nerve angiomatosis and multiple soft tissue tumors Am J Surg Pathol 19:1325-1332, 1995 Proteus syndrome – multiple lipomas JAAD 52:834-838, 2005; BJD 151:953-960, 2004; AD 140:947-953, 2004; JAMA 285:2240-2243, 2001; lipomatosis with hemihypertrophy Textbook of Neonatal Dermatology, p.466, 2001 Pseudohypoparathyroidism – dry, scaly, hyperkeratotic puffy skin; multiple subcutaneous osteomas, collagenoma BJD 143:1122-1124, 2000 Pseudoxanthoma elasticum – multiple calcified cutaneous nodules Am J Med 31:488-489, 1961 Steatocystoma multiplex JAAD 43:396-399, 2000; AD Syphilol 36:31-36, 1937

Intravenous drug abuse – foreign body granulomas or non-specific non-granulomatous inflammation; panniculitis BJD 150:1-10, 2004

Churg-Strauss syndrome JAAD 47:209-216, 2002; Mayo Clinic Proc 52:477-484, 1977 Diffuse neonatal hemangiomatosis Ped Derm 14:383-386, 1997 Generalized lymphangiomatosis Ped Derm 15:296-298, 1998 Glomus tumors – multiple or plaque type; hemi-facial JAAD 45:239-245, 2001; Ped Derm 18:223-226, 2001; AD 127:1717-1722, 1991 Henoch-Schönlein purpura Leung, Robson, 2000 Kaposiform hemangioendothelioma JAAD 38:799-802, 1998 Kimura’s disease JAAD 43:905-907, 2000 Klippel-Trenaunay-Weber syndrome Polyarteritis nodosa, systemic; cutaneous (livedo with nodules) – painful or asymptomatic red or skin-colored multiple nodules with livedo reticularis of feet, legs, forearms face, scalp, shoulders, trunk Ped Derm 15:103-107, 1998; AD 130:884-889, 1994; JAAD 31:561-566, 1994; JAAD 31:493-495, 1994 Takayasu’s arteritis NEJM 349:160-169, 2003

Bowel arthritis dermatitis syndrome – erythema nodosum-like lesions AD 135:1409-1414, 1999; Cutis 63:17-20, 1999; JAAD 14:792-796, 1986; Mayo Clin Proc 59:43-46, 1984; AD 115:837-839, 1979 Chronic granulomatous disease JAAD 36:899-907, 1997; suppurative cutaneous granulomas in chronic granulomatous disease (Microascus cinereus) Clin Inf Dis 20:110-114, 1995; also Aspergillus, Candida, Paecilomyces, Exophiala dermatiditis, Acremonium strictum, Sarcinosporon inkin Complement deficiency – low serum complement with SLE-like illness Clin Res 22:416 (Abstract ), 1974 Dermatitis herpetiformis – prurigo nodularis-like lesions J Eur Acad Dermatol Venereol 16:88-89, 2002 Dermatomyositis – panniculitis which may ulcerate and form sinuses BJD 128:451-453, 1993; AD 119:336-344, 1983; nodules and plaques on arms, thighs, buttocks, abdomen with lipoatrophy AD 127:1846-1847, 1991; JAAD 23:127-128, 1990; erythema nodosum Rook p.2560, 1998, Sixth Edition Fogo selvagem (endemic pemphigus) – prurigo nodularis-like lesions JID 107:68-75, 1996; JAAD 32:949-956, 1995 Lupus erythematosus – lupus panniculitis (lupus profundus) – thighs, buttocks, arms, breasts, face Rook p.2451, 1998, Sixth Edition; AD 122:576, 1986; AD 103:231-242, 1971; LE hypertrophicus et profundus – mimicking thrombophlebitis J Rheumatol 16:1400, 1989; chilblain lupus – chilblain-like lesions of legs Rook p.2455, 1998, Sixth Edition Pemphigoid nodularis JAAD 53:S101-104, 2005; JAAD 29:293-299, 1993; JAAD 21:1099-1104, 1989 Rheumatoid arthritis – neutrophilic lobular panniculitis JAAD 45:325-361, 2001; J R Soc Med 84:307-308, 1991; palisaded neutrophilic granulomatous dermatitis of rheumatoid arthritis (rheumatoid neutrophilic dermatosis)

JAAD 47:251-257, 2002; JAAD 45:596-600, 2001; AD 133:757-760, 1997; AD 125:1105-1108, 1989 Sjögren’s syndrome – plasma cell panniculitis J Cutan Pathol 23:170-174, 1996; red nodules of legs JAAD 48:311-340, 2003

Leukemia cutis Dermatol Therapy 18:104-116, 2005

Corticosteroids – post-steroid panniculitis Ped Derm 5:92-93, 1988; AD 90:387-391, 1964; topical corticosteroidinduced infantile gluteal granuloma Clin Exp Dermatol 6:23-29, 1981 Enfuvirtide – injection site reaction JAAD 49:826-831, 2003 Erythema nodosum – drug-induced erythema nodosum – sulfonamides, other antibiotics, analgesics, antipyretics, birth control pill, granulocyte colony-stimulating factor, all-trans retinoic acid Rook p.3393, 1998, Sixth Edition Furosemide – Sweet’s like JAAD 21:339-343, 1989 G-CSF – Sweet’s syndrome, pyoderma gangrenosum Ped Derm 17:205-207, 2000; neutrophilic dermatosis of legs and buttocks Ped Derm 18:417-421, 2001 Imatinib (Gleevec) BJD 149:678-678, 2003; imatinib-associated Sweet’s syndrome AD 141:368-370, 2005 Immunization granuloma Interleukin-2 injections – lobular panniculitis Br J Cancer 66:698-699, 1992 Iododerma Australas J Dermatol 28:119-122, 1987 Leukocyte colony stimulating factors AD 130:77-81, 1994 Meperidine AD 110:747-750, 1974 Pentazocine AD 110:747-750, 1974 Sulindac-induced pancreatitis JAAD 45:325-361, 2001 Vasculitis secondary to various drugs; propylthiouracil nodular vasculitis Cutis 49:253-255, 1992

Aluminum hypersensitivity – vaccination sites Ghatan p.252, 2002, Second Edition Aspartame (Nutra-Sweet) – lobular panniculitis JAAD 24:298-300, 1991 Contact dermatitis, irritant Mercury ingestion JAAD 37:131-133, 1997 Paraffinoma Plast Reconstr Surg 65:517-524, 1980 Povidone panniculitis AD 116:704-706, 1980 Silicone granuloma AD 117:366-367, 1981 Zyderm test site

Abscess Acanthamebiasis in AIDS JAAD 42:351-354, 2000; Arch Int Med 157:569-572, 1997; AD 131:1291-1296, 1995; Cutis 56:285-287, 1995; NEJM 331:85-87, 1994; Ped Inf Dis 11:404-407, 1992 Actinomycetoma – Nocardiopsis dassonvillei AD 121:1332-1334, 1985 Actinomycosis – panniculitis J Cutan Pathol 16:183-193, 1989

AIDS – neutrophilic eccrine hidradenitis in HIV infection J Dermatol 25:199-200, 1998; Int J Dermatol 35:651-652, 1996 Alternaria chartarum BJD 142:1261-1262, 2000 Aspergillosis – primary cutaneous aspergillosis JAAD 38:797-798, 1998 Bacterial sepsis Bartonellosis – verruga peruana; bacillary angiomatosis Tyring p.228-229, 2002 Bacillus species in subacute combined immunodeficiency JAAD 39:285-287, 1998 Bartonella bacilliformis – Oroya fever with verruga peruana – red papules in crops become nodular, hemangiomatous or pedunculated; face, neck, extremities, mucosal lesions Ann Rev Microbiol 35:325-338, 1981 Borrelia burgdorferi – nodular panniculitis J Infect Dis 160:596-597, 1992; Lyme borreliosis – acrodermatitis chronica atrophicans – red to blue nodules or plaques; tissue-paper-like wrinkling; pigmented; poikilodermatous; hands, feet, elbows, knees BJD 121:263-269, 1989: Int J Derm 18:595-601, 1979 Brucellosis – red papulonodules of legs (erythema nodosum-like lesions) JAAD 48:474-476, 2003; Cutis 63:25-27, 1999; AD 117:40-42, 1981 Candida sepsis – papules and nodules with pale centers Am J Dermatopathol 8:501-504, 1986; JAMA 229:1466-1468, 1974; septic panniculitis in infancy Textbook of Neonatal Dermatology, p.425, 2001 Cat scratch disease – erythema nodosum Pediatrics 81:559-561, 1988 Chagas’ disease – reactivation chagoma AD 139:104-105, 2003 Chromomycosis JAAD 8:1-16, 1983 Coccidioidomycosis – red nodule of arm JAAD 49:944-949, 2003 Corynebacterium jeikeium Cryptococcosis – erythema nodosum-like (cryptococcal panniculitis) AD 112:1734-1740, 1976; Arch Int Med 136:670-677, 1976; BJD 74:43-49, 1962 Cysticercosis Dematiaceous fungal infections in organ transplant recipients – all lesions on extremities

Alternaria Bipolaris hawaiiensis Exophiala jeanselmei, E. spinifera, E. pesciphera, E. castellani Exserohilum rostratum Fonsacaea pedrosoi Phialophora parasitica

Fascioliasis – Fasciola hepatica (fluke parasite) – eosinophilic panniculitis JAAD 42:900-902, 2000 Fusarium – red nodule with central pallor Sabouradis 17:219-223, 1979; dark nodules of leg Clin Inf Dis 32:1237-1240, 2001 Fusobacterium – septic panniculitis in infancy Textbook of Neonatal Dermatology, p.425, 2001 Gnathostomiasis – red migratory swellings BJD 145:487-489, 2001; JAAD 33:825-8, 1995; eosinophilic migratory panniculitis (larva migrans profundus – Gnathostoma doloresi or G. spinigerum) JAAD 11:738-740, 1984 Gonococcemia Am J Med Sci 260:150-159, 1970 Hepatitis B – erythema nodosum JAAD 9:602-603, 1983; hepatitis A – nodular panniculitis Cutis 32:543-547, 1983; polyarteritis nodosa Herpes simplex – chronic HSV infection in AIDS – red nodule resembling basal cell carcinoma JAAD 36:831-833, 1997

Histoplasmosis Diagnostic Challenges V 77-79, 1994; AD 121:914-916, 1985 Insect bite reactions Klebsiella species – septic panniculitis in infancy Textbook of Neonatal Dermatology, p.425, 2001 Leishmaniasis Clinics in Dermatology 14:425-431, 1996; post-kala-azar dermal leishmaniasis Leprosy – lepromatous leprosy including erythema nodosum leprosum (vasculitis) AD 138:1607-1612, 2002; AD 111:1575-1580, 1975; subcutaneous lepromas Rook p.1224-1227, 1998, Sixth Edition Lobomycosis – Loboa loboi – legs, arms, face Rook p.1359-1360, 1998, Sixth Edition Loiasis Meningococcemia, chronic BJD 153:669-671, 2005; Rev Infect Dis 8:1-11, 1986; acute Am J Med Sci 260:150-159, 1970 Microascus cinereus in chronic granulomatous disease J Clin Inf Dis 20:110-114, 1995 Milker’s nodules Tyring p.58, 2002 Mucormycosis – Mucor racemosa Mycetoma JAAD 49:S170-173, 2003; JAAD 32:311-315, 1995; Cutis 49:107-110, 1992; Australas J Dermatol 31:33-36, 1990; JAAD 6:107-111, 1982; Sabouraudia 18:91-95, 1980; AD 99:215-225, 1969

Eumycetoma Pseudoallescheria boydii Madurella mycetomatis Madurella grisea Exophiala jeanselmei Pyrenochaeta romeroi Leptosphaera senegaliensis Curvularia lunata Neotestudina rosatii Aspergillus nidulans Aspergillus flavus Fusarium spp. Cylindrocarpon spp.