Xeroderma pigmentosum or XP is classified as a rare skin disease.
In the United States, 1 in 250,000 people are affected. In some other parts of the world such as Japan, the frequency can be 1 in 40,000. Individuals with this skin disorder do not have the ability to repair the damage to skin cells caused by exposure to ultraviolet light. The result is the presence of growths in the skin that can become malignant. Younger individuals are affected more and detection often occurs at one or two years of age. Affected individuals develop skin cancer at a young age, which can lead to death at an early age.
There are 7 to 8 classes of this skin condition. The symptoms are: the presence of many freckles at a young age, irregular pigmented patches or dark spots on the skin, very dry or crusty/scaly skin, thinning of the skin layer, the presence of several solar keratoses, a severe sunburn with blistering that lasts for several weeks even though sun exposure was not severe, the premature aging of the skin – in particular the lips, eyes, and mouth regions, the presence of spidery blood vessels on the skin, and limited hair growth on the chest and leg regions. Many people, up to 80 percent, suffering from xeroderma have very sensitive eyes and ultraviolet radiation exposure causes ocular problems such as conjunctivitis. In general, when these symptoms are noticed in young individuals in sun exposed areas of the skin, then a visit to a physician is required so that a proper diagnosis can be made.
Treatment of this skin condition involves the proper monitoring of any skin growths that appear to ensure they do not develop into skin cancer and to remove problematic growths quickly when necessary. This type of diligent monitoring will require regular and frequent visits to a doctor or dermatologist. The use of oral retinoids can help to decrease the occurrence of skin cancer in people suffering from xeroderma pigmentosum. Certain topical treatments can also be used to treat the skin keratoses that develop.
There is no cure for this skin problem. The best treatment is to limit exposure to damaging sunrays as much as possible. This involves staying out of the sun. When in the sun, high protection sunscreens (high SPF with proper ultraviolet protection) should be used without exception. Exposed skin should be covered up.
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