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CREST syndrome

Disease definition

CREST syndrome is a subtype of limited cutaneous systemic sclerosis (lcSSc; see this term) whose name is an acronym for the cardinal clinical features of the syndrome: calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia.

ORPHA:90290

  • Synonym(s):
    • Calcinosis-Raynaud phenomenon-esophageal involvement-sclerodactyly-telangiectasia syndrome
  • Prevalence: 1-9 / 100 000
  • Inheritance: Multigenic/multifactorial or Not applicable 
  • Age of onset: Adult
  • ICD-10: M34.1
  • OMIM: 181750
  • UMLS: C0206138
  • MeSH: D017675
  • GARD: 12430
  • MedDRA: 10011380

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