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J Korean Soc Pediatr Nephrol. 2006 Apr;10(1):52-57. Korean. Case Report.
Bae K , Lee MK , Lee JH , Park YS , Huh J .
Department of Pediatrics, Asan Medical Center, University of Ulsan, College of Medicine, Seoul, Korea. yspark@amc.seoul.kr
Department of Pathology, Asan Medical Center, University of Ulsan, College of Medicine, Seoul, Korea.
Abstract

Kimura's disease is a rare chronic inflammatory disease of unknown etiology which appears primarily in young Asian males as non-tender subcutaneous swellings in the head and neck region. Histologic characteristics are the presence of lymphoid follicles, vascular proliferation and infiltration of eosinophils. Peripheral eosinophilia and elevated serum IgE are frequently combined. Systemic steroid therapy with surgical excision is the mainstay of treatment, though recurrence after surgery or discontinued steroid treatment is common. It has been known that about 16% of the cases are associated with renal diseases, particularly nephrotic syndrome. We present an 8-year-old boy with a past history of steroid-responsive, infrequently relapsing nephrotic syndrome who developed right buccal swelling and peripheral eosinophilia during the remission state. He has been managed with surgical resection, steroid and cyclosporine due to multiple recurrences.

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