J Korean Ophthalmol Soc.  2018 Nov;59(11):1071-1076. 10.3341/jkos.2018.59.11.1071.

IgG4-related Ophthalmic Disease Associated with Adult Xanthogranulomatous Disease

• Lee S
• Chung S
• Heo J
• Lew H

• KMID: 2426369
• DOI: http://doi.org/10.3341/jkos.2018.59.11.1071

Abstract

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.

Keyword

Adult xanthogranulomatous disease; IgG4-related ophthalmic disease; Xanthogranuloma

MeSH Terms

Adult*
Azathioprine
Biopsy
Blepharoplasty
Cholecystectomy
Diagnosis
Diagnosis, Differential
Eyelids
Giant Cells
Histiocytes
Humans
Immunoglobulin G
Immunoglobulins
Magnetic Resonance Imaging
Male
Orbit
Pathology
Plasma Cells
Prednisolone
Azathioprine
Immunoglobulin G
Immunoglobulins
Prednisolone

Figure

• 

  Figure 1 Clinical photographs of the patient's eyelid with the diagnosis of adult xanthogranulomatous disease. The patient visited clinic with bilateral eyelid swelling and yellowish mass (A, B). Four years later, he was re-visited with the same symptoms (C, D).

• 

  Figure 2 Orbital computed tomography on the first visit demonstrated bilateral soft tissue swelling with no bone invasions (A, B). Coronal image (A), Axial image (B), magnetic resonance imaging on 4 years later demonstrated bilateral soft tissue swelling with no bone invasions (C, D). T2 axial image show diffuse swelling with heterogenous signal intensities at bilateral lacrimal glands (C). T1 axial image show heterogenous soft tissue swelling at bilateral eyelids and preseptal areas (D).

• 

  Figure 3 Histologic findings of orbital mass on the first excisional biopsy. (A) Variable germinal centers and nodular lymphoid infiltrates in the lacrimal gland and the touton giant cells (arrow) and many foamy histiocytes in the subcutaneous layer (Hematoxylin and eosin [H&E] stain, ×200). (B, C) Immunohistochemical stain of orbital mass 4 years later, immunoglobulin G4 [IgG4]+/IgG+ plasma cell ratio of 40–50% and 59 IgG4+ plasma cells per high power field (HPF). IgG plasma cells (×100) (B), IgG4 plasma cells (×100) (C). Histologic findings of orbital mass on relapse (D). Diffuse infiltration of foamy histiocytes (arrow) and a few lymphoplasma cells (dash arrow) (H&E stain, ×100). (E, F) Immunohistochemical stain of orbital mass on relapse, IgG4+/IgG+plasma cell ratio of 10–20%and 22 IgG4+ plasma cells per HPF. IgG plasma cells (×100) (E), IgG4 plasma cells (×100) (F).

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