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Kimura disease

Orpha number ORPHA482
Synonym(s) Eosinophilic lymphogranuloma
Prevalence <1 / 1 000 000
Inheritance Unknown
Age of onset Adult
ICD-10
  • D21
  • I89.8
  • L92.8
ICD-O -
OMIM -
UMLS
  • C0033838
MeSH
  • D000796
MedDRA
  • 10048640
SNOMED CT
  • 399894006

Summary

Kimura's disease, a rare entity in the West but endemic in Asia, manifests as solitary or multiple subcutaneous nodules, primarily located in the cervical region. They are often accompanied by local adenopathies and/or salivary gland hypertrophy. Histologically, the lesions are characterized by hyperplastic lymphoid tissue, an inflammatory infiltrate rich in eosinophils and a proliferation of postcapillary venules. Hypereosinophilia in the blood and elevated levels of circulating IgE are found. A nephrotic syndrome must be systematically sought. The etiology of this chronic inflammatory disease is unknown. An aberrant immune reaction to an unknown antigenic stimulus has been suggested. Treatment consists of surgical excision of the lesion(s) and corticotherapy is prescribed for relapsing forms and when renal involvement is present. The prognosis is good and no malignant transformation has ever been observed. Other drug classes have been tried with some success.

Expert reviewer(s)

  • Pr Olivier BLETRY
  • Dr Claire LARROCHE

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Detailed information

Review article
  • EN (2005,pdf)
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