ICD-11 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: XPA
to XPG, and XP variant (XPV). The severity of the clinical manifestations and the
age of onset are extremely variable and are in part dependent on exposure to sunlight
and the complementation group.;
ICD-11 synonym : XP - [xeroderma pigmentosum]; atrophoderma pigmentosum;
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: XPA
to XPG, and XP variant (XPV). The severity of the clinical manifestations and the
age of onset are extremely variable and are in part dependent on exposure to sunlight
and the complementation group.