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Xeroderma pigmentosa

• Overview
• Symptoms
• Treatment
• Prevention

Definition:

Xeroderma pigmentosa is a rare condition passed down through families in which the skin and the tissue covering the eye is extremely sensitive to the ultraviolet part of sunlight.

Causes, incidence, and risk factors:

Ultraviolet light -- such as that found in sunlight -- damages the genetic material (DNA) in skin cells. Normally, the body repairs this damage. But in persons with xeroderma pigmentosa, the body does not fix the damage. As a result, the skin gets very thin and patches of varying color (splotchy pigmentation) appear.

The condition also causes spidery blood vessels in the skin ( telangiectasia ) and skin cancer. Skin cancer often occurs before the child is 5 years old.

References:

Abeloff MD, Armitage JO, Niederhuber JE, Kastan MB, McKena WG. Clinical Oncology . 3rd ed. Orlando, Fl: Churchill Livingstone; 2004.

Kumar V, Abbas AK, Fausto N. Robbins and Cotran Pathologic Basis of Disease . 7th ed. St. Louis, Mo: WB Saunders; 2005.

• Review Date: 4/12/2007
• Reviewed By: Kevin Berman, MD, PhD, Associate, Atlanta Center for Dermatologic Disease, Atlanta, GA. Review provided by VeriMed Healthcare Network.

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