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Erdheim-Chester disease

Disease definition

Erdheim-Chester disease (ECD), a non-Langerhans form of histiocytosis, is a multisystemic disease characterized by various manifestations such as skeletal involvement with bone pain, exophthalmos, diabetes insipidus, renal impairment and central nervous system (CNS) and/or cardiovascular involvement.

ORPHA:35687

  • Synonym(s): -
  • Prevalence: Unknown
  • Inheritance: Not applicable 
  • Age of onset: Adult
  • ICD-10: D76.3
  • OMIM: -
  • UMLS: C0878675
  • MeSH: D031249
  • GARD: 6369
  • MedDRA: 10060801

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