ICD-11 classes
20 Developmental anomalies
Multiple developmental anomalies or syndromes
LD27 — Syndromes with skin or mucosal anomalies as a major feature
EC40 — Pseudoxanthoma elasticum

ICD-11 EC40 — Pseudoxanthoma elasticum

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.

The diagnosis includes nothing.

The diagnosis excludes nothing.

It has no clarifying diagnoses.

The diagnosis is coded elsewhere:

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