MANAGEMENT OF A YOUNG PATIENT WITH XERODERMA-PIGMENTOSUM

Xeroderma pigmentosum is a group of rare autosomal recessive disorders with defective DNA repair that provide insight into the basic mechani sm of carcinogenesis. It is the best human model linking clinical abno rmalities and neoplasia to carcinogen exposure. We describe a patient with xeroderma pigmentosum and numerous basal cell carcinomas, squamou s cell carcinomas, and melanomas treated with radiation therapy, Mohs micrographic surgery, dermabrasion, and isotretinoin prophylaxis.