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Modern Rheumatology Volume 29, 2019 - Issue 2
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Case Reports

Ocular adnexal marginal zone lymphoma arising in a patient with IgG4-related ophthalmic disease

Kenji NishidaDepartment of Pathology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan,
,
Yuka SogabeDepartment of Ophthalmology, Mitoyo General Hospital, Kan-Onji, Japan,
,
Ayako MakiharaDepartment of Dermatology, Mitoyo General Hospital, Kan-Onji, Japan,
,
Akemi SenooDepartment of Dermatology, Mitoyo General Hospital, Kan-Onji, Japan,
,
Hisanori MorimotoDepartment of Internal Medicine, Mitoyo General Hospital, Kan-Onji, Japan, and
,
Mai TakeuchiDepartment of Pathology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan,
,
Yuka GionDepartment of Pathology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan,
,
Tadashi YoshinoDepartment of Pathology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan,
&
Yasuharu SatoDepartment of Pathology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan, ;Division of Pathophysiology, Okayama University Graduate School of Health Sciences, Okayama, JapanCorrespondence[email protected]
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Pages 383-387 | Received 02 Feb 2016, Accepted 21 Jul 2016, Published online: 11 Aug 2016
 
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Abstract

A 41-year-old man was diagnosed with immunoglobulin G4-related disease (IgG4-RD) in both eyelids 4 years ago and exhibited good response to steroid therapy. However, rapid swelling of the right eyelid lesion was recently observed. As IgG4-RD progression was suspected, biopsy was performed. Although the histology was consistent with IgG4-RD, the infiltrating large atypical lymphoid cells showed immunoglobulin light-chain restriction and IgH gene rearrangement. Consequently, he was diagnosed with extranodal marginal zone lymphoma with abundant IgG4-positive cells.

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