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Kimura disease

ORPHA482
Synonym(s) Eosinophilic lymphogranuloma
Prevalence <1 / 1 000 000
Inheritance Unknown
Age of onset Adult
ICD-10
  • I89.8
OMIM -
UMLS
  • C0033838
MeSH
  • D000796
MedDRA
  • 10048640

Summary

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 a 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.


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