Kimura Disease Presenting as Unilateral Proptosis

Abstract

Purpose: Kimura disease is a rare chronic inflammatory disease that usually presents with painless subcutaneous nodules at the head and neck area, but rarely occurs around the eyes. We report unique case of Kimura disease involving the orbit and arm, which has not been reported in Korea.Case summary: A 16-year-old boy presented with exophthalmos and eyelid swelling of right eye over the last 2 years. Orbital magnetic resonance imaging (MRI) showed hypertrophy of extraocular muscles of right eye. He also complained of multiple painless masses of the left forearm that persisted during the same period. The MRI showed hypertrophy of extraocular muscles of right eye and multiple lymphadenopathy along the brachial artery of the left elbow. Excisional biopsy of the left arm mass and lymph nodes was performed and the lesions were diagnosed as Kimura disease involving the dermal nodule and lymph nodes. The patient was managed with oral corticosteroid and cyclosporine and showed remarkable improvement of proptosis and hypertrophy of extraocular muscles.Conclusions: We report a rare case of Kimura disease causing hypertrophy of extraocular muscles which was successfully treated with immunosuppressive agents.