EC20.2

ICD-11 MMS 2026-01

Hereditary acantholytic dermatoses

Description

A group of heritable disorders characterised by epidermal acantholysis and loss of epidermal integrity.

Index terms

  • Hereditary acantholytic dermatoses
  • Darier disease
  • Darier disease (MIM 124200)
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  • Darier-White disease
  • Dyskeratosis follicularis
  • Keratosis follicularis
  • Acrokeratosis verruciformis of Darier
  • Hailey-Hailey disease
  • Benign familial pemphigus
  • Gougerot-Hailey-Hailey disease

Inclusions

  • Darier disease
  • Hailey-Hailey disease

Sibling codes

  • EC20.0 – Non-syndromic ichthyosis
  • EC20.1 – Hereditary skin peeling
  • EC20.3 – Hereditary palmoplantar keratodermas
  • EC20.Y – Other specified genetic disorders of keratinisation

Foundation entity

Hereditary acantholytic dermatoses – http://id.who.int/icd/entity/248560941

Part of: EC20: Genetic disorders of keratinisation › 14: Diseases of the skin