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

Abstract

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.

Kimura’s Disease With Recurrent Bilateral Lacrimal Gland Involvement in a Male Japanese Child Successfully Treated With Cyclosporine A

“…These histological features may overlap with those of idiopathic orbital in ammation [8,12] or ALHE [1,2]. Notably, some cases of KD occurring in the left lacrimal gland with increased serum levels of IgG4, mimicking IgG4related disease, have been reported [14,15]. In the current case, we made a differential diagnosis of IgG4related disease, despite normal IgG4 levels and lack of IgG4 expression in the lacrimal gland specimen.…”

Kimura’s disease with recurrent bilateral lacrimal gland involvement in a male Japanese child successfully treated with cyclosporine A

“…These histological features may overlap with those of idiopathic orbital inflammation [ 8 , 12 ] or ALHE [ 1 , 2 ]. Notably, some cases have been reported of KD occurring in the left lacrimal gland with increased serum levels of IgG4, mimicking IgG4-related disease [ 14 , 15 ]. In the current case, we made an initial differential diagnosis of IgG4-related disease, despite normal IgG4 levels and lack of IgG4 expression in the lacrimal gland specimen.…”

Three unusual histopathological presentations of angiolymphoid hyperplasia with eosinophilia

“…We have not found any previous case of IgG4‐related disease associated with ALHE. However, there are cases of Kimura disease imitating IgG4‐related disease . Moreover, some authors have even considered the increase in IgG4‐positive plasma cells as an epiphenomenon of Kimura disease .…”

“…However, there are cases of Kimura disease imitating IgG4-related disease. 44,45 Moreover, some authors have even considered the increase in IgG4-positive plasma cells as an epiphenomenon of Kimura disease. 46 There is also a case of ALHE in the literature that showed a prominent fibrotic component and a granulomatous reaction with numerous giant cells.…”