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Gazzetta Medica Italiana Archivio per le Scienze Mediche 1999 April;158(2):45-9

Copyright © 1999 EDIZIONI MINERVA MEDICA

language: Italian

An unclear syndrome: Kimura’s disease

Cau C.

Azienda Ospedaliera S. Giovanni-Addolorata - Roma, DEA


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Kimura’s disease is a chronic inflammatory disorder of unknown etiology, presenting usually as painless subcutaneous swellings in the head and neck region or in the salivary glands. It was first described in the chinese literature in 1937 under the designation “eosinophilic hyperplasic lymphogranuloma”. This entity became more widely known as Kimura’s disease in the Japanese literature. The pathogenesis of Kimura’s disease is not clear. An atopic reaction to a variety of agents appears to be an alternative hypothesis. Histopathological findings are: proliferation of lymphofollicular structure and of granulation tissue with eosinophil infiltration. In additions systemic findings including peripheral eosinophilia and elevated serum IgE levels almost always present Kimura’s disease can be confused clinically and histologically with angiolymphoid hyperplasia with eosinophilia. In some cases the kidney, eyelids, orbit, and tympanic membrane are involved. The clinical course is progressive but often becomes stationary after a few years. Malignant transformation has never been documented. For the initial diagnosis, excisional biopsy is important for the exclusion of malignant lymohoma, histiocytosis X, angiolymphoid hyperplasia with eosinofilia and other reactive lymphadenopathies. Nonetheless fine needle aspiration cytology may be useful in the diagnosis of recurrent lesions of Kimura’s disease and may spare the patient from repeated biopsies. Many methods of treatment such as surgical excision, steroids, radiation have been tried. However recurrence is common but prognosis is never unfavourable.

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