ABSTRACT
Paget's disease is a malignant tumor mainly deriving from apocrine glands. Two types of Paget's disease have been described: the most frequent mammary form and the extramammary form. Mammary Paget's disease almost exclusively appears in adult women affected by breast cancer and involves the nipple and the areola of the same breast. Extramammary Paget's disease is mainly seen in body areas rich in apocrine glands, such as the anogenital and axillary areas, and is caused by a coexisting carcinoma only in less than half of cases. On clinical examination Paget's disease appears as a single erythematous/eczematous plaque that may be pigmented or nonpigmented. In nonpigmented forms dermoscopic examination reveals milky-red areas, irregular linear vessels, shiny white lines and scales, while in pigmented forms additional criteria may be seen, such as light brown diffuse pigmentation and peppering-like blue-gray dots.
