J Korean Ophthalmol Soc JKOS Journal of the Korean Ophthalmological Society 0378-6471 2092-9374 The Korean Ophthalmological Society 10.3341/jkos.2018.59.11.1071 Case Report IgG4-related Ophthalmic Disease Associated with Adult Xanthogranulomatous Disease Lee Seunghyun MD 1 Chung Sokjoong MD 1 Heo Jinhyung MD PhD 2 Lew Helen MD PhD 1 Department of Ophthalmology, CHA Bundang Medical Center, Seongnam, Korea. Department of Pathology, CHA Bundang Medical Center, Seongnam, Korea. Address reprint requests to Helen Lew, MD, PhD. Department of Ophthalmology, CHA Bundang Medical Center, #59 Yatap-ro, Bundang-gu, Seongnam 13496, Korea. Tel: 82-31-780-5330, Fax: 82-31-780-5336, eye@cha.ac.kr 11 2018 16 11 2018 59 11 1071 1076 12 07 2018 20 08 2018 21 10 2018 ©2018 The Korean Ophthalmological Society 2018 The Korean Ophthalmological Society This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. Purpose

To report a case of immunoglobulin G4 (IgG4)-related ophthalmic disease associated with adult xanthogranulomatous disease.

 Case summary

A 38-year-old male with a history of cholecystectomy visited our clinic for bilateral periorbital swelling. Histopathology of the orbital biopsy showed diffuse infiltration of foamy histiocytes with Touton giant cells and lymphoid follicles, with a diagnosis of adult-onset xanthogranuloma. After excisional biopsy, he was treated with azathioprine and prednisolone. Four years after treatment, he again visited the clinic due to bilateral, yellowish eyelid masses. Serological examinations were all nonspecific findings, except for elevation of IgG and IgG4 levels. Magnetic resonance imaging showed bilateral symmetric soft tissue enlargement with slightly heterogeneous T1/T2 isosignal intensity, with contrast enhancement at the superolateral aspect of extraconal spaces. Excisional biopsy and blepharoplasty were performed. Immunohistochemical sections showed that the IgG4+/IgG plasma cell ratio was 10–20% and the IgG4 plasma cell count was 22/high power field (HPF). His past sections of 2013 from the pathology department were again stained and showed that the IgG4+/IgG plasma cell ratio was 40–50% and the IgG4 plasma cell count was 59/HPF. Thus, he was definitely diagnosed with IgG4-related ophthalmic disease.

 Conclusions

If there is recurrent eyelid swelling, IgG4-related ophthalmic disease should be considered as a differential diagnosis. And the patient with adult xanthogranulomatous disease can be diagnosed with IgG4-related ophthalmic disease.

 Adult xanthogranulomatous disease IgG4-related ophthalmic disease Xanthogranuloma

The authors have no conflicts to disclose.

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