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Diffuse cutaneous systemic sclerosis

Disease definition

Diffuse cutaneous systemic sclerosis (dcSSc) is a subtype of Systemic Sclerosis (SSc; see this term) characterized by truncal and acral skin fibrosis with an early and significant incidence of diffuse involvement (interstitial lung disease, oliguric renal failure, diffuse gastrointestinal disease, and myocardial involvement).

ORPHA:220393

  • Synonym(s):
    • Diffuse cutaneous systemic scleroderma
    • Progressive cutaneous systemic scleroderma
    • Progressive cutaneous systemic sclerosis
  • Prevalence: -
  • Inheritance: Multigenic/multifactorial or Not applicable 
  • Age of onset: Adult
  • ICD-10: M34.0
  • OMIM: -
  • UMLS: -
  • MeSH: -
  • GARD: 9751
  • MedDRA: -

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