Xeroderma pigmentosum

ICD-10 Code Q82.1

Xeroderma pigmentosum

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).

Also known as angioma pigmentosum atrophicum, atrophoderma pigmentosum, Kaposi dermatosis, Kaposi disease, melanosis lenticularis progressiva, pigmented epitheliomatosis, xeroderma of Kaposi, xeroderma pigmentosum syndrome, XP — per MONDO

Also identified as

Affected Organs

Organ Involvement Source
Integumental system Disease Has Associated Anatomic Site NCIT · CC BY 4.0
Skin of body Disease Has Primary Anatomic Site NCIT · CC BY 4.0