14Diseases of the skin Genetic or developmental disorders affecting the skin Genetic disorders affecting dermal collagen, elastin or other matrix proteins
EC40
category
Pseudoxanthoma elasticum
Definition 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.
Also indexed as
PXE - [Pseudoxanthoma elasticum] Pseudoxanthoma elasticum
Nearby in Genetic disorders affecting dermal collagen, elastin or other matrix proteins
International Classification of Diseases, Eleventh Revision (ICD-11), World Health Organization (WHO) 2019/2021, https://icd.who.int/browse11. Licensed under Creative Commons Attribution-NoDerivatives 3.0 IGO (CC BY-ND 3.0 IGO).
Release 2026-01.
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This page reproduces WHO classification content for reference. It is not
medical advice and must not be used to diagnose or treat. For coding
decisions, consult the official release and your national coding guidelines.
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