Genodermatoses with profound malignant potential.

Abstract

There are a number of heritable disorders that have some association with skin cancer. Xeroderma pigmentosum, nevoid basal cell carcinoma syndrome, and familial melanoma and dysplastic nevi are three disorders associated with an extremely high rate of cutaneous malignancy. There is no known cure for these disorders, thus, patients and families need information about the disease process, treatment options, and guidelines aimed at prevention and early detection of skin cancer.