Kimura disease is a rare chronic inflammatory disorder of unknown etiology. It presents as a deep, subcutaneous mass in the head and neck region and is frequently associated with regional lymphadenopathy or salivary gland involvement.
The disease was first described in the Chinese literature as eosinophilic hyperplastic lymphogranuloma but became widely known as Kimura disease after Kimura et al reported similar cases under the title on the unusual granulation combined with hyperplastic changes of lymphatic tissue.
Over the years, there has been considerable confusion between Kimura disease and angiolymphoid hyperplasia with eosinophilia (ALHE). Indeed, a number of early reports used the terms Kimura disease and ALHE synonymously. It is now thought, however, that the two diseases represent separate entities with distinctive clinic and histologic features.
Kimura disease is considered much more prevalent in young males of Asian lineage. The nodular lesions are deep-seated in subcutaneous tissue and clinically may mimic a neoplasm. One of the important clinicopathologic features is its frequent association with regional lymph node and/or major salivary glands involvement. Indeed, isolated lymphadenopathy might be the only initial presentation of Kimura disease.
Clinical synopsis
blood eosinophilia
high concentration of IgE
cervical lymphadenopathy
Microscopy
eosinophilic epithelioid granulomatous reaction (12094384)
multiple epithelioid granulomas with central eosinophilic abscesses and necrosis
macrophages and giant cells phagocyte the apoptotic eosinophils at the edge of the granulomas
follicular hyperplasia
eosinophilic infiltrate
proliferation of postcapillary venules
References
Chen H, Thompson LD, Aguilera NS, Abbondanzo SL. Kimura disease: a clinicopathologic study of 21 cases. Am J Surg Pathol. 2004 Apr;28(4):505-13. PMID: 15087670