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 mechanism of carcinogenesis. It is the best human model linking clinical abnormalities and neoplasia to carcinogen exposure. We describe a patient with xeroderma pigmentosum and numerous basal cell carcinomas, squamous cell carcinomas, and melanomas treated with radiation therapy, Mohs micrographic surgery, dermabrasion, and isotretinoin prophylaxis.
Leal-Khouri
S
S
Department of Medicine, Washington University School of Medicine, St. Louis, Missouri.
Hruza
G J
GJ
Hruza
L L
LL
Martin
A G
AG
D000293:Adolescent; D002280:Basal Cell Carcinoma / Q000628:therapy; D002294:Carcinoma, Squamous Cell / Q000628:therapy; D003131:Combined Modality Therapy; D005153:Facial Neoplasms / Q000628:therapy*; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008545:Melanoma / Q000628:therapy; D009364:Neoplasm Recurrence, Local; D009378:Neoplasms, Multiple Primary / Q000628:therapy; D012878:Skin Neoplasms / Q000628:therapy*; D014983:Xeroderma Pigmentosum / Q000628:therapy*