ICD-11 classes
14 Diseases of the skin
Inflammatory dermatoses
Urticaria, angioedema and other urticarial disorders
EB03 — Syndromes with urticarial reactions or angioedema
4A60.1 — Cryopyrin-associated periodic syndromes
ICD-11 4A60.1 — Cryopyrin-associated periodic syndromes
CAPS is an autoinflammatory disease associated with gain of function changes in the cryopyrin protein, resulting in inflammasome activation and enhanced IL1 beta production. This results in clinical signs and symptoms of inflammation in the form of rash, fever, joint and eye symptoms with increased acute phase reactants.
It includes 1 item.
- Cryopyrinopathies
The diagnosis excludes nothing.
It has no clarifying diagnoses.
The diagnosis is coded elsewhere: