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Kimura disease

Disease definition

Kimura disease is a benign and chronic inflammatory disorder of unknown etiology, occurring mainly in Asian countries (very rarely in Western countries) and predominantly affecting young men, that usually presents with solitary or multiple non-tender subcutaneous masses in the head and neck region (in particular the preauricular and submandibular area) and/or generalized painless lymphadenopathy, often with salivary gland involvement. Characteristic laboratory findings include blood eosinophilia and markedly elevated serum immunoglobulin E (IgE) levels. It is often associated with autoinflammatory disorders (i.e. ulcerative colitis, bronchial asthma) and a co-existing renal disease.


Classification level: Disorder
  • Synonym(s):
    • Eosinophilic lymphogranuloma
  • Prevalence: <1 / 1 000 000
  • Inheritance: Unknown 
  • Age of onset: Adult
  • ICD-10: I89.8
  • OMIM: -
  • UMLS: C0033838
  • MeSH: D000796
  • GARD: 6835
  • MedDRA: 10048640
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