All the Damage Without the Burn

A diagnosis of xeroderma pigmentosum should be considered in a child presenting with progressive lentigines on photoexposed sites, even without severe and prolonged sunburn.

Patients with xeroderma pigmentosum (XP) have a considerably elevated risk for skin cancer due to a congenital defect in the repair of ultraviolet (UV) radiation–induced DNA damage. Mutations causing XP fall into eight complementation groups: XP-A to XP-G (classical XP) and XP variant (XP-V). Sunburn reactions have been considered critical to the development of XP in all patients. However, it is not clear whether individuals with differing complementation groups all have similar sunburn reactions.

These investigators examined sunburn reactions in XP patients and correlated reactions with complementation group. Using a newly developed sunburn severity score in 60 patients seen at an XP center in the U.K., the researchers found unusually high …

Reviewing Author

Hensin Tsao, MD, PhD

Disclosures

Consultant / advisory board Lubax; WorldCare Clinical

EquityLubax

Grant / Research support NIH; Department of Defense; American Skin Association; Piramal

Editorial boardsBritish Journal of Dermatology; Journal of the American Academy of Dermatology; Journal of Investigative Dermatology

Leadership positions in professional societies American Academy of Dermatology (Chair, Skin Cancer and Melanoma Committee); American Board of Dermatology (Director)