LD27.6

ICD-11 MMS 2026-01

Genetic lipodystrophy

Description

Genetic lipodystrophies represent a heterogeneous group of rare diseases characterised by a generalised or localised loss of body fat (lipoatrophy).

Exclusions from above levels

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

Child codes

  • LD27.60 – Congenital generalised lipodystrophy
  • LD27.6Z – Genetic lipodystrophy, unspecified

Sibling codes

  • LD27.0 – Ectodermal dysplasia syndromes
  • LD27.1 – Xeroderma pigmentosum
  • 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.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

Genetic lipodystrophy – http://id.who.int/icd/entity/1166232738

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