Disease name

키무라 병
 Kimura disease

Prevalence

<1 / 1 000 000

Inheritance

미상

Age of onset

성인기

ICD 10 code

D21, I89.8, L92.8

Synonym

Eosinophilic lymphogranuloma

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.