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cryopyrin-associated periodic syndrome

Medical Definition

A group of rare autosomal dominant diseases, commonly characterized by atypical urticaria (hives) with systemic symptoms that develop into end-organ damage. the atypical hives do not involve t-cell or autoantibody. cryopyrin-associated periodic syndrome includes three previously distinct disorders: familial cold autoinflammatory syndrome; muckle-wells syndrome; and cinca syndrome, that are now considered to represent a disease continuum, all caused by nlrp3 protein mutations.
Related Codes (1)
Code
Description
Billable
Details
M04.2Cryopyrin-associated periodic syndromes

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