Abstract

Xeroderma pigmentosum (XP) is a rare autosomal recessive disease of DNA repair characterized by severe ultraviolet (UV) sensitivity resulting in a 10,000-fold increased risk for skin cancer. Symptoms include freckle-like pigmentation in sun exposed skin before age 2 years, severe burns after minimal sun exposure (50% of patients) and skin cancers in children. XP is also considered a premature aging disease because of its features such as: dry skin, increased sun-induced freckling in young patients, higher incidence of skin cancers in young patients and premature menopause.

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