Immunoglobulin G4-related Sclerosing Disease of the Breast and Skin in a Male Patient
HKY Tam, LWY Chan, GKF Tam, MWS Chau, APY Tang
Hong Kong J Radiol 2017;20:e1-6
DOI: 10.12809/hkjr1715360
Immunoglobulin G4 (IgG4)–related disease affects multiple organs and is characterised by an increase in serum IgG4 concentration, IgG4-positive plasma cells, lymphocytic infiltration, inflammation, and fibrosis. Its aetiology remains unknown. We describe the first male patient with simultaneous involvement of the breast and skin. Mammography, ultrasonography, and magnetic resonance imaging revealed an index mass with multiple nodules in the left breast and skin nodules. Cancer was excluded radiologically, and image-guided biopsy of the breast and skin lesions showed many plasma cells within the lesions immunohistochemically positive for IgG4. The patient also had a raised serum IgG4 titre. The diagnosis of IgG4-related sclerosing disease was confirmed. We describe the imaging and pathological features of this entity in breast and skin. It should be considered a differential diagnosis of a breast lesion and can mimic malignancy. Biopsy for histological confirmation is advocated. It has good response to systemic steroid therapy, and unnecessary surgery can be avoided.
Authors’ affiliations:
HKY Tam, GKF Tam: Department of Radiology, North District Hospital, Sheung Shui, Hong Kong
LWY Chan: Department of Radiology, Tseung Kwan O Hospital, TKO, Hong Kong
MWS Chau: Department of Clinical Pathology, North District Hospital, Sheung Shui, Hong Kong
APY Tang: Department of Diagnostic Radiology, Alice Ho Miu Ling Nethersole Hospital, Tai Po, Hong Kong
中文摘要
一位男性患者乳房皮膚免疫球蛋白G4相關性硬化症疾病
譚家盈、陳慧儀、譚國輝、周慧璇、鄧佩儀
免疫球蛋白G4（IgG4）相關疾病能影響多個器官，其特徵是血清IgG4濃度增加、IgG4陽性漿細胞增加、淋巴細胞滲透、炎症和纖維化。其病原仍然未知。我們描述首例男性乳腺和皮膚的IgG4相關疾病。乳房X線照相、超聲檢查和磁共振成像顯示主瘤與多個結節在左乳房和皮膚。影像學檢查排除癌症，影像學引導乳房和皮膚組織活檢顯示病變內許多漿細胞免疫組織化學IgG4呈陽性，患者的血清IgG4滴度亦上升。我們描述此疾病的乳房和皮膚影像和病理特徵。它可表現類似惡性腫瘤，是乳腺病變的一個鑑別診斷。建議組織活檢查斷診。此病對類固醇治療有良好的反應，應避免不必要的手術。