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Muckle-Wells syndrome

Disease definition

Muckle-Wells syndrome (MWS) is an intermediate form of cryopyrin-associated periodic syndrome (CAPS; see this term) and is characterized by recurrent fever (with malaise and chills), recurrent urticaria-like skin rash, sensorineural deafness, general signs of inflammation (eye redness, headaches, arthralgia/myalgia) and potentially life-threatening secondary amyloidosis (AA type).

ORPHA:575

  • Synonym(s):
    • Neutrophilic urticaria
  • Prevalence: Unknown
  • Inheritance: Autosomal dominant 
  • Age of onset: Childhood, Neonatal, Infancy
  • ICD-10: E85.0
  • OMIM: 191900
  • UMLS: C0268390
  • MeSH: -
  • GARD: 8472
  • MedDRA: 10064569

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