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Xeroderma pigmentosum

 

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About 200 people in the United States, or two to four persons in about 1 million live births, suffer from xeroderma pigmentosum, a rare genetic defect that causes extreme sensitivity to the sun's ultraviolet rays. Ultraviolet light disrupts normal cell functioning in people who have the disorder, causing cancerous cell changes. Although there are various degrees of the disorder, most persons with xeroderma pigmentosum acquire severe sunburn after any sun exposure, although using sunscreen and layering clothes sometimes protects against the sun's negative effects.

xeroderma pigmentosum

Dr. Kenneth H. Kraemer, research dermatologist at the National Cancer Institute in Bethesda, says patients with the disease have a thousandfold increase in their chances of getting skin cancer compared to the average person. The first skin cancer may develop before a person is 10 years old, and many other skin cancers may occur in the future.

Xeroderma Pigmentosum Society (www.xps.org) is a nonprofit organization that sponsors Camp Sundown, a nighttime camp held in the summer that is open to xeroderma pigmentosum patients and their families. Sufferers of other light-sensitive conditions also are welcome.

 

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