ICD-11 classes
14 Diseases of the skin
Genetic and developmental disorders affecting the skin
DNA instability syndromes affecting the skin
LD27.1 — Xeroderma pigmentosum

ICD-11 LD27.1 — Xeroderma pigmentosum

Xeroderma pigmentosum (XP) is a rare genodermatosis characterised 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.

The diagnosis includes nothing.

The diagnosis excludes nothing.

It has no clarifying diagnoses.

The diagnosis is coded elsewhere:

• Inherited cancer-predisposing syndromes #2829
• Syndromes with skin or mucosal anomalies as a major feature #23324