ABSTRACT
Nevoid basal cell carcinoma syndrome (NBCCS) or Gorlin-Goltz syndrome is an autosomal dominant genodermatosis mainly characterized by the presence of multiple and early onset basal cell carcinomas (BCCs), odontogenic keratocysts and palmoplantar pits.
Cutaneous BCCs clinically look as pearly, flesh-colored, or pigmented papules, nodules or plaques and may appear in unusual body sites. These BCCs do not differ in their dermoscopic aspect from those arising in nonsyndromic patients. They usually show arborizing vessels, ulceration, fine telangiectasias and pigmented structures as blue-gray ovoid nests, dots and globules, maple leaflike areas and spoke-wheel structures.
Acral pits appear as pale, flesh-colored, pink or red punctiform depressions, mainly localized on palms. On dermoscopy, they appear as small red or pink depressed lesions with sharp borders.
Treatments of BCCs in NBCCS include excisional surgery, laser therapy, electrocoagulation, cryosurgery, photodynamic therapy, topical imiquimod, 5-fluorouracil and new oral hedgehog pathway inhibitors.
