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Management of xeroderma pigmentosum.

Xeroderma pigmentosum is an autosomal recessive disease associated with a defect in DNA repair from damage by ultraviolet light. It is characterized by the development of cutaneous malignancies in childhood and by death from the local or systemic complications thereof by the third decade of life. The head and the neck are the most frequently affected areas. Management involves sunlight avoidance and prompt biopsy of any new skin lesion. Due to the chronic nature of the disease, it is necessary to excise the least amount of tissue consistent with tumor removal in order to preserve skin for future procedures. In some patients, radiation therapy and/or certain chemotherapeutic agents are abnormally cytotoxic and must be utilized with caution.