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Drug rash with eosinophilia and systemic symptoms

Disease definition

A rare hypersensitivity reaction characterized by a generalized skin rash, fever, eosinophilia, lymphocytosis and visceral involvement (hepatitis, nephritis, pneumonitis, pericarditis and myocarditis) and, in some patients, reactivation of human herpes virus 6. Onset usually occurs 2-6 weeks after administration of the causal medication and is most frequently associated with anticonvulsants and sulfonamides but other medications (allopurinol, cyclosporine, azathioprine, gold salts and antiviral agents) have also been implicated.

ORPHA:139402

Classification level: Disorder
  • Synonym(s):
    • DRESS syndrome
    • Drug reaction eosinophilic systemic syndrome
  • Prevalence: Unknown
  • Inheritance: Not applicable 
  • Age of onset: All ages
  • ICD-10: T78.4
  • OMIM: -
  • UMLS: C1142139  C3541994
  • MeSH: -
  • GARD: -
  • MedDRA: 10058919
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