EC40

ICD-11 MMS 2026-01

Pseudoxanthoma elasticum

Description

Pseudoxanthoma elasticum (PXE) is an inherited connective tissue disorder characterised by progressive calcification and fragmentation of elastic fibres in the skin, retina, and arterial walls.

Index terms

  • Pseudoxanthoma elasticum
  • PXE - [Pseudoxanthoma elasticum]

Sibling codes

  • EC4Y – Other specified genetic disorders affecting dermal matrix proteins

Foundation entity

Pseudoxanthoma elasticum – http://id.who.int/icd/entity/1516160852

Part of: 14: Diseases of the skin