A case of refractory Kimura disease with a buccal bulky mass successfully?treated with low-dose cyclosporine A: Report and?review?of?the literature

摘要

Kimura disease (KD) is a chronic inflammatory disorder that presents as painless lymphadenopathy or subcutaneous masses, most predominantly in the head and neck region. Peripheral eosinophilia and elevated serum IgE levels are the characteristics of the disease. Promoted helper type 2 (Th2) responses are suggested to be the mechanism underlying KD. Although the disease has a benign course, relapse often occurs after the first-line therapy, which includes surgical excision, radiotherapy, and topical steroids. Recently, cyclosporine A (CsA) for the treatment of recurrent KD has been reported. Here, we describe a patient with recurrent KD after surgical and medium-dose steroid therapy who was successfully treated with low-dose (2 mg/kg/d) CsA. We also review the literature related to CsA treatment for KD.