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xeroderma pigmentosum
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xeroderma pigmentosum
n.
(Medicine) genetic disease of dry skin that becomes discolored and is characterized by unusual sensitivity to sunlight
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Xeroderma pigmentosum
Xeroderma pigmentosum, or XP, is an autosomal recessive genetic disorder of DNA repair in which the ability to repair damage caused by ultraviolet (UV) light is deficient. In extreme cases, all exposure to sunlight must be forbidden, no matter how small. Multiple basal cell carcinomas (basaliomas) and other skin malignancies frequently occur at a young age in those with XP. In fact, metastatic malignant melanoma and squamous cell carcinoma are the two most common causes of death in XP victims. This disease involves both sexes and all races, with an incidence of 1:250,000 and a gene frequency of 1:200. XP is roughly six times more common in Japanese people than in other groups.
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xeroderma pigmentosum
Noun
1. a rare genetic condition characterized by an eruption of exposed skin occurring in childhood and photosensitivity with severe sunburn; inherited as a recessive autosomal trait in which DNA repair processesm are defective so they are more likely to chromosome breaks and cancers when exposed to ultraviolet light
(hypernym) xeroderma, xerodermia
| NCI Dictionary of Cancer Terms | Download this dictionary |
xeroderma pigmentosum
A genetic condition characterized by a sensitivity to all sources of ultraviolet radiation.
A Service of the National Cancer Institute.
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XERODERMA PIGMENTOSUM
XERODERMA PIGMENTOSO
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