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Eosinophilic fasciitis

Disease definition

A rare idiopathic inflammatory myopathy that is characterized by inflammation and thickening of the fascia, usually associated with peripheral eosinophilia. It presents during adulthood with symmetrical and painful swelling of mainly the extremities that progressively become indurated. Fatigue, disabling cutaneous fibrosis, myositis and arthritis may also be observed.

ORPHA:3165

Classification level: Disorder
  • Synonym(s):
    • Diffuse fasciitis with eosinophilia
    • Shulman syndrome
  • Prevalence: Unknown
  • Inheritance: Unknown 
  • Age of onset: Adult
  • ICD-10: M35.4
  • ICD-11: 4A43.4
  • OMIM: 226350
  • UMLS: C0264005
  • MeSH: C562487
  • GARD: 6351
  • MedDRA: 10014954
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