Orbital Mass With Features of Both Kimura Disease and Immunoglobulin G4-Related Disease

Kimura disease has been known as a chronic inflammatory disease of unknown etiology, since the first report in 1937 and characterized by an increased eosinophil fraction in peripheral blood, increased serum immunoglobulin E (IgE) levels, and lymphoid granuloma with eosinophilic infiltration on histopathological examination. The authors present a 30-year-old man who visited their clinic with a 3-month history of protrusion and conjunctival injection of the right eye. Surgical mass resection was performed, and initial histopathology revealed Kimura disease. Additional immunohistochemical examination revealed IgG4-related disease in another hospital, which showed more than 80 IgG4-positive plasma cells per high-power field and an IgG4+/IgG+ ratio more than 40%. There are histopathological similarities, and several reports have suggested a relationship between Kimura disease and IgG4-related disease. Thus, it is difficult to classify them as completely different diseases. Kimura disease and IgG4 can be considered a part of a clinical spectrum of abnormal immune reactions. Accepted for publication March 25, 2018. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Sung Chul Kim, M.D., HanGil Eye Hospital, Incheon 21388, South Korea. E-mail: hapto@daum.net © 2018 by The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc., All rights reserved.