Cryopyrin-associated periodic syndrome
From IDWiki
Background
- Mutation in NLRP3 gene
- One of the periodic fever syndrome
Clinical Manifestations
- Three different clinical manifestations:
- Familial cold-associated syndrome (FCAS)
- Episodes of fever, rashes, arthralgias, usually triggered by exposure to cold
- Muckle-Wells syndrome (MWS)
- Episodes of fever, but with migrating urticaria-like lesions, ocular abnormalities, and progressive sensorineural deafness, and possibly amyloidosis
- Chronic infantile neurological cutaneous articular syndrome (CINCA syndrome)
- Familial cold-associated syndrome (FCAS)
