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Kimura’s disease with recurrent bilateral lacrimal gland involvement in a male Japanese child successfully treated with cyclosporine A
Allergy, Asthma & Clinical Immunology ( IF 2.6 ) Pub Date : 2021-05-17 , DOI: 10.1186/s13223-021-00549-y
Keisuke Sugimoto , Takuji Enya , Yuichi Morimoto , Rina Oshima , Kohei Miyazaki , Mitsuru Okada

Kimura’s disease (KD) is a rare chronic inflammatory disease of unknown etiology. Clinically, KD is characterized by nodular subcutaneous masses, that are typically localized to the neck and head. Involvement of the lacrimal glands and limbs is uncommon and seldom reported. We report a case of a 4-year-old Japanese boy presenting with bilateral upper eyelid swelling with nodular subcutaneous lesions and peripheral eosinophilia. Based on clinical, histopathological, and laboratory findings, the patient was diagnosed with KD. An itchy subcutaneous mass on the left arm developed at the age of 14 years. Treatment with steroids was effective. However, as the steroids were tapered after the patient developed side effects, the masses relapsed within a few months. Treatment with cyclosporine A was then initiated, which led to an improvement of clinical features and serial levels of cytokines. We report a rare case of KD with a peculiar clinical presentation. The patient responded well to treatment with cyclosporine A.

中文翻译:

木环氏病伴双侧泪腺反复累及一名日本男性儿童,成功用环孢霉素A治疗

木村病(KD)是一种病因不明的罕见慢性炎症性疾病。临床上,KD的特征是结节性皮下肿块,通常位于颈部和头部。很少有泪腺和四肢受累的报道。我们报告了一例4岁的日本男孩与双侧上睑红肿,结节性皮下病变和周围嗜酸性粒细胞增多症的情况。根据临床,组织病理学和实验室检查结果,该患者被诊断为KD。左臂发痒的皮下肿物在14岁时发展。类固醇治疗有效。但是,由于在患者出现副作用后类固醇逐渐减少,所以肿块在几个月内复发了。然后开始用环孢霉素A治疗,这导致临床特征和细胞因子系列水平的改善。我们报告了罕见的KD病例,并伴有特殊的临床表现。该患者对环孢霉素A的治疗反应良好。
更新日期:2021-05-17
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