Immunoglobulin G4-related Sclerosing Disease of the Breast and Skin in a Male Patient

Full Article

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