LD28.10

ICD-11 MMS 2026-01

Ehlers-Danlos syndrome, classical type

Description

Ehlers-Danlos syndrome, classic type is a type of Ehlers-Danlos syndromes (EDS), a heterogeneous group of hereditary connective tissue diseases characterised by joint hyperlaxity, cutaneous hyperelasticity and tissue fragility, and is characterised by the following major clinical diagnostic criteria: hyperextensible skin, atrophic cutaneous scars due to tissue fragility and joint hyperlaxity.

Index terms

  • Ehlers-Danlos syndrome, classical type
  • Ehlers-Danlos syndrome type 1
  • Ehlers-Danlos syndrome type 2

Exclusions from above levels

  • EC40 – Pseudoxanthoma elasticum
  • EE41.0 – Cutis laxa
  • http://id.who.int/icd/entity/733825440 – Inborn errors of metabolism

Sibling codes

  • LD28.1Y – Other specified types of Ehlers-Danlos syndrome
  • LD28.1Z – Ehlers-Danlos syndrome, unspecified

Foundation entity

Ehlers-Danlos syndrome, classical type – http://id.who.int/icd/entity/1724920772

Allowed postcoordination axes

Part of: LD28.1: Ehlers-Danlos syndrome › LD28: Syndromes with connective tissue involvement as a major feature › 20: Developmental anomalies