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Xeroderma pigmentosum

ORPHA:910· ICD-10 Q82.1

Definition

Xeroderma pigmentosum (XP) is a rare genodermatosis characterized by extreme sensitivity to ultraviolet (UV)-induced changes in the skin and eyes, and multiple skin cancers. It is subdivided into 8 complementation groups, according to the affected gene: classical XP (XPA to XPG) and XP variant (XPV).

Prevalence
1-9 / 1 000 000
Inheritance
Autosomal recessive
Age of onset
All ages