LD27.1

ICD-11 MMS 2026-01

Xeroderma pigmentosum

Description

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.

Index terms

  • Xeroderma pigmentosum
  • XP - [xeroderma pigmentosum]
  • atrophoderma pigmentosum
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  • Xeroderma pigmentosum A
  • XPA - [xeroderma pigmentosum A]
  • Xeroderma pigmentosum B
  • XPB - [xeroderma pigmentosum B]
  • Xeroderma pigmentosum C
  • XPC - [xeroderma pigmentosum C]
  • Xeroderma pigmentosum D
  • XPD - [xeroderma pigmentosum D]
  • Xeroderma pigmentosum E
  • XPE - [xeroderma pigmentosum E]
  • Xeroderma pigmentosum F
  • XPF - [xeroderma pigmentosum F]
  • Xeroderma pigmentosum G
  • XPG - [xeroderma pigmentosum G]

Exclusions from above levels

  • http://id.who.int/icd/entity/733825440 – Inborn errors of metabolism

Sibling codes

  • LD27.0 – Ectodermal dysplasia syndromes
  • LD27.2 – Syndromic ichthyosis
  • LD27.3 – Genetic syndromes with hypertrichosis
  • LD27.4 – Genetic syndromes affecting nails
  • LD27.5 – Genetic hamartoneoplastic syndromes affecting the skin
  • LD27.6 – Genetic lipodystrophy
  • LD27.Y – Other specified syndromes with skin or mucosal anomalies as a major feature
  • LD27.Z – Syndromes with skin or mucosal anomalies as a major feature, unspecified

Foundation entity

Xeroderma pigmentosum – http://id.who.int/icd/entity/1243068849

Part of: LD27: Syndromes with skin or mucosal anomalies as a major feature › 20: Developmental anomalies