Kimura’s disease of the lacrimal gland mimicking IgG4-related orbital disease

BMC Ophthalmology

Case Report

Jinru Li¹  Xin Ge¹  Jianmin Ma¹  Jing Li²  Ming Li³ 

[1] Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing Ophthalmology and Visual Sciences Key Laboratory, No. 1 Dongjiaominxiang Street, Dongcheng District, 100730, Beijing, China;Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing Ophthalmology and Visual Sciences Key Laboratory, No. 1 Dongjiaominxiang Street, Dongcheng District, 100730, Beijing, China;Beijing Institute of Ophthalmology, Beijing Tongren Hospital, Capital Medical University, Beijing, China;Department of Pathology, Beijing Tongren Hospital, Capital Medical University, Beijing, China;

关键词: Kimura’s disease;    IgG4-related disease;    Lacrimal gland;   

DOI  :  10.1186/1471-2415-14-158

 received in 2014-10-09, accepted in 2014-12-11,  发布年份 2014

来源: Springer

【 摘 要 】

BackgroundKimura’s disease (KD) is a rare and benign chronic inflammatory soft tissue disorder of unknown origin, which predominantly inflicts young male adults in Asia. IgG4-related disease is a new disease concept, established this century and characterized by fibrosis and sclerosis of the involved organs, with infiltration of IgG4-positive plasma cells. These two kinds of diseases share similar characteristics, which may complicate their diagnosis.Case presentationA 47-year-old Chinese man presented to our Department of Ophthalmology with a 26-month history of painless swelling and redness left upper eyelid. Surgical excisions of the left lacrimal gland were performed. A histopathology examination showed follicular hyperplasia with reactive germinal centres and eosinophilic infiltration involving the interfollicular areas as well as proliferation of post capillary venules, all signs of Kimura disease. Immunohistochemical analysis of the cells demonstrated positive staining for CK, Vimentin, CD3, CD4, CD20, CD21, CD117, CD5, CD8, CD23, IgG and IgG4 (30 per high-power field) and negative staining for CD10 and CD34. Some ophthalmologists in our department questioned whether the histological and immunohistochemical findings were also compatible with features of IgG4-related diseases. There was no sign of recurrence during the twelve months of regular follow-up.ConclusionKimura’s disease may present with high serum IgG4 levels, which may be an epiphenomenon related to chronic antigen exposure. As clinical doctors, especially ophthalmologists, we should recognize the possibility of the occurrence of increased serum levels of IgG4 in Kimura’s disease to ensure correct diagnosis.

【 授权许可】

Unknown   
© Li et al.; licensee BioMed Central Ltd. 2014. This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.