Kimura's disease: clinical characteristics, management and outcome of 20 cases from China.

Abstract

To explore the clinical characteristics, diagnosis and the therapeutic effect of Kimura's disease (KD). Clinical data of 20 patients with pathologically confirmed KD admitted to Peking University People's Hospital from June 2000 to June 2019 were analysed. A total of 20 confirmed KD patients were enrolled in the study, 18 male and 2 female, with age-onset ranging from 2 to 58 years. The masses appear as focal, painless, and immovable with an unclear boundary. The most common predilection is head-neck region (n=15, 75%). 15 patients showed peripheral blood eosinophilia. 14 of 14 patients presented with increased serum IgE level. The prominent pathological characteristic is marked lymphoid hyperplasia accompanied by various degrees of vascular hyperplasia and eosinophil infiltration. Among the 20 patients, 12 experienced recurrence of disease after treatment (surgical resection alone: 9/9; oral corticosteroids combined with immunosuppressants: 1/3; surgical resection followed by oral corticosteroids combined with immunosuppressants: 2/6). KD should be considered when the patient presents with head-neck swellings and lymphadenopathy, accompanied by an increase of IgE and eosinophil. Compared with surgery alone, combined therapy seems to be a promising treatment option to reduce the recurrence rate.