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Xeroderma Pigmentosum

Also called: XP, Melanosis Lenticularis, Kaposi Disease

- Summary
- About xeroderma pigmentosum
- Types and differences
- Risk factors and causes
- Signs and symptoms
- Diagnosis methods
- Treatment options
- Prevention methods for complications
- Ongoing research
- Questions for your doctor

Reviewed By:
Kimberly Bazar, M.D., AAD

Prevention Methods for complications

Many people with xeroderma pigmentosum (XP) die by age 20 from skin cancer. However, if a person is diagnosed early, has no severe neurological complications (e.g., dementia), is protected from ultraviolet (UV) radiation and regularly checked for early signs of skin cancer, a normal lifespan may be possible.

XP patients must avoid UV radiation at all times to protect their skin. If sun exposure cannot be entirely avoided, it should be kept to a minimum and patients should wear wide-brimmed hats, protective clothing with long sleeves, use sunscreen with sun protection factor (SPF) 50 and wear dark, UV-absorbent sunglasses to protect the eyes. The rays of the sun are the strongest, and thus cause the most damage, between the hours of 10 a.m. and 4 p.m. If it is not possible to avoid those hours, patients should seek shade under a tree, umbrella or tent. Sometimes patients may opt to change their lifestyle to completely avoid sun exposure by only going outside at night.

When indoors, XP patients should be protected from unfiltered (bare) fluorescent light bulbs and sunlight coming through windows. A special UV protective film is available to coat windows in homes and cars.

Other steps that XP patients can take to help prevent skin cancer include:

  • Avoid other sources of UV radiation. Besides the sun, ultraviolet rays are also found in the light given off by germicidal lamps, artificial sunlamps (such as those used in tanning salons) and mercury-vapor lamps.

  • Avoid cigarette smoke. Laboratory tests indicate that smoking or exposure to tobacco smoke can also result in damage to the skin cells of XP patients in the same way that UV exposure does.

  • Check medications. Some prescription drugs increase a person’s sensitivity to sunlight, putting them at greater risk for sunburn. Common medications that increase sensitivity include diuretics, antibiotics and nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen.

It is recommended that all patients with XP have regular checkups (every three to six months) with their dermatologist and ophthalmologist (eye-care specialist) to monitor their condition. XP patients should also perform frequent self-examinations of the eyes, scalp, ears, mouth, tongue, nostrils and all other areas of the skin, even those not regularly exposed to sunlight (e.g., buttocks), and promptly report any suspicious spots or growths to their physician. XP patients should also have an annual neurological examination, including a hearing evaluation, until the age of 20.

Ongoing research on xeroderma pigmentosum

The genes responsible for most types of xeroderma pigmentosum (XP) have been identified. Researchers throughout the United States, Europe and Japan are currently trying to correct the deoxyribonucleic acid (DNA, the molecule that encodes genetic information) repair defect in laboratory-grown cells from patients with the genetic disorder. The U.S. National Institutes of Health is also conducting clinical studies on skin cancer prevention with oral medications and evaluating patients with unusual features.

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Review Date: 02-08-2007
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