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J. David Osguthorpe, MD; Pearon Lang, MD

Arch Otolaryngol Head Neck Surg. 1987;113(3):292-294.

Abstract
• 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.

(Arch Otolaryngol Head Neck Surg 1987;113:292-294)

Author Affiliations
From the Departments of Otolaryngology and Communicative Sciences (Dr Osguthorpe) and Dermatology (Dr Lang), Medical University of South Carolina, Charleston.

Footnotes
Accepted for publication Oct 6, 1986.

Read before the Spring Meeting of the American Academy of Facial Plastic and Reconstructive Surgery, Palm Beach, Fla, May 10, 1986.

Reprint requests to Department of Otolaryngology and Communicative Sciences, Medical University of South Carolina, 171 Ashley Ave, Charleston, SC 29425-2242 (Dr Osguthorpe).

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