This Article  • Full text  • PDF  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citation map  • Contact me when this article is cited  Related Content  • Similar articles in this journal  Topic Collections  • Dermatologic Disorders  • Physical Examination  • Neoplasms  • Diagnosis  • Alert me on articles by topic  Social Bookmarking   What's this?

W. Harris Green, MD; Steven Q. Wang, MD; Armand B. Cognetta Jr, MD

Arch Dermatol. 2009;145(8):910-915.

Background  Xeroderma pigmentosum (XP) is an autosomal recessive disorder characterized by a defect in DNA repair and subsequent increased frequency of cutaneous malignant neoplasms, including melanoma. In patients with XP, patient and family education and aggressive UV radiation protection are the primary means of skin cancer prevention. An important secondary measure in decreasing morbidity and mortality in these patients involves early detection of skin cancers, particularly melanomas.

Observations  We describe a 39-year-old woman with XP who developed 38 primary melanomas along with 6 squamous cell carcinomas and 70 basal cell carcinomas over a 23-year period. During this time, a 3-fold management approach of total-body cutaneous examination, total-body photography, and dermoscopy was used in the care of the patient. The thickest melanoma had a Breslow thickness of 1.07 mm, and the mean Breslow thickness of her detected melanomas was 0.18 mm. The ratio of benign to malignant biopsied suspicious melanocytic lesions during 23 years of follow-up was 0.9:1. All melanomas were treated using wide local excision, and she had no evidence of local or in-transit metastases of any of her malignant neoplasms at the most recent follow-up examination.

Conclusion  Monthly follow-up using total-body cutaneous examinations, total-body photography, and dermoscopy is an important 3-fold secondary management technique for this unique patient, allowing early detection of her melanomas.

Author Affiliations: Division of Dermatology, Brody College of Medicine at East Carolina University, Greenville, North Carolina (Dr Green); Dermatology Division, Memorial Sloan-Kettering Cancer Center, New York, New York (Dr Wang); and Dermatology Associates of Tallahassee, Tallahassee, Florida (Dr Cognetta).

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter     What's this?