Soft Tissue Mass as Primary Manifestation of IgG4 Related Disease

Fariba  Abbasi; Ata  Abbasi; Mohammad Reza  Pashaei; Khadijeh  Azimi; Pegah  Saedifar; Amir  Nezafat

doi:10.18502/acta.v64i3.21546

Fariba Abbasi Department of Pathology, Solid Tumor Research Center, Cellular and Molecular Medicine Institute, Urmia University of Medical Sciences, Urmia, Iran
Ata Abbasi Department of Pathology, Faculty of Medicine, Urmia University o Medical Sciences, Urmia, Iran
Mohammad Reza Pashaei Department of Gastroenterology, Patient Safety Research Center, Clinical Research Institute, Urmia University of Medical Sciences, Urmia, Iran
Khadijeh Azimi Department of Gastroenterology, Patient Safety Research Center, Clinical Research Institute, Urmia University of Medical Sciences, Urmia, Iran
Pegah Saedifar Department of Pathology, Faculty of Medicine, Urmia University o Medical Sciences, Urmia, Iran
Amir Nezafat Department of Pathology, Faculty of Medicine, Urmia University o Medical Sciences, Urmia, Iran

DOI: https://doi.org/10.18502/acta.v64i3.21546

Keywords: IgG4-related disease; Soft tissue; Mass; Unusual presentation; Single organ

Abstract

IgG4- related disease is a systemic fibroinflammatory syndrome characterized by tumefactive enlargement of affected organ , diffuse lymphoplasma cells infiltration with a large number of IgG4 producing plasma cells and sclerotic changes. The most commonly involved organs are pancreas, lacrimal and salivary glands. Patients show good response to glucocorticoid therapy and despite its relapsing and remitting course, patients have good prognosis. We report an unusual presentation of IgG4- related disease manifested as isolated soft tissue mass of hand which is an extremely rare manifestation of this disorder.