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Juvenile dermatomyositis

Disease definition

An early-onset form of dermatomyositis (DM), a systemic, autoimmune inflammatory muscle disorder with vasculopathy, characterized by proximal and symmetrical muscle weakness, evocative skin lesions, and systemic manifestations. Vasculopathy occurs in the skin, muscle (mainly in the perifascicular area), and sometimes in the intestinal tissue.

ORPHA:93672

Classification level: Disorder
  • Synonym(s):
    • Juvenile DM
  • Prevalence: Unknown
  • Inheritance: Not applicable 
  • Age of onset: Infancy, Childhood, Adolescent
  • ICD-10: M33.0
  • OMIM: -
  • UMLS: C0263666  C2931785
  • MeSH: C538250
  • GARD: 6805
  • MedDRA: 10008521
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