LD28.1

ICD-11 MMS 2026-01

Ehlers-Danlos syndrome

Description

Ehlers-Danlos syndrome (EDS) is a heterogeneous group of inherited disorders of connective tissue, principally collagen, that range in severity from mild joint hypermobility to life-threatening fragility of soft tissue and vasculature.

Exclusions from above levels

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

Child codes

  • LD28.10 – Ehlers-Danlos syndrome, classical type
  • LD28.1Y – Other specified types of Ehlers-Danlos syndrome
  • LD28.1Z – Ehlers-Danlos syndrome, unspecified

Sibling codes

  • LD28.0 – Marfan syndrome or Marfan-related disorders
  • LD28.2 – Genetically-determined cutis laxa
  • LD28.Y – Other specified syndromes with connective tissue involvement as a major feature
  • LD28.Z – Syndromes with connective tissue involvement as a major feature, unspecified

Foundation entity

Ehlers-Danlos syndrome – http://id.who.int/icd/entity/1122707206

Allowed postcoordination axes

Part of: LD28: Syndromes with connective tissue involvement as a major feature › 20: Developmental anomalies