Genodermatoses with malignant potential.

Disease-associated malignancy may occur in several inherited conditions with dermatologic manifestations. These genodermatoses include nevoid basal cell carcinoma syndrome, Cowden's disease, multiple endocrine neoplasia syndromes, neurofibromatosis and Peutz-Jeghers syndrome. Although most of these genodermatoses are rare, recognition of their cutaneous features can facilitate early diagnosis of the systemic condition and surveillance for neoplasm. Appropriate evaluation of the patient's family, as well as genetic counseling, should be performed when the diagnosis of a genodermatosis with malignant potential is established.