LD27.03

ICD-11 MMS 2026-01

Hidrotic ectodermal dysplasia, Clouston type

Description

Clouston syndrome (or hidrotic ectodermal dysplasia) is an inherited disorder characterised by the clinical triad of nail dystrophy, alopecia, and palmoplantar hyperkeratosis.

Index terms

  • Hidrotic ectodermal dysplasia, Clouston type
  • Clouston syndrome (MIM 129500)

Exclusions from above levels

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

Sibling codes

  • LD27.00 – Incontinentia pigmenti
  • LD27.01 – Cronkhite-Canada syndrome
  • LD27.02 – Hypohidrotic ectodermal dysplasia
  • LD27.0Y – Other specified ectodermal dysplasia syndromes
  • LD27.0Z – Ectodermal dysplasia syndrome, unspecified

Foundation entity

Hidrotic ectodermal dysplasia, Clouston type – http://id.who.int/icd/entity/1811429982

Part of: LD27.0: Ectodermal dysplasia syndromes › LD27: Syndromes with skin or mucosal anomalies as a major feature › 20: Developmental anomalies