Perifollicular granulomas with IgG4 plasmacytosis: A case report and review of literature

Abstract

Perifollicular granuloma is a unique histologic feature and whether it is associated with immunoglobulin G4 (IgG4)-related disease is controversial. We report a case of a 38-year-old man who presented with worsening left eye pain, proptosis, tearing, gritty sensation, blurred vision and multiple lymphadenopathy. An axillary lymph node resection showed reactive follicular and interfollicular lymph node hyperplasia, and increased eosinophils and plasma cells (at least 80% of IgG(+) plasma cells were positive for IgG4). A distinct feature was the presence of multifocal, perifollicular histiocytic granulomas, which formed a wreath around the entire follicles. The human herpes virus 8 was not detected by immunohistochemistry. In addition, an extensive panel of special stains, immunohistochemistry, and flow cytometry was negative for lymphoma, fungal, or mycobacterial infection. The findings were suggestive of IgG4-related sclerosing disease-associated lymphadenopathy. Further laboratory testing showed a significant increase of serum immunoglobulin E (> 23000 IU/mL) and slight increase of total IgG, but normal serum IgG4. Even though perifollicular granuloma is a nonspecific histopathologic feature and can be seen in other diseases, such as nodular lymphocyte predominant Hodgkin lymphoma, IgG4-related lymphadenopathy should be listed in the differential diagnoses of benign reactive lymph nodes, especially when perifollicular granuloma and plasmacytosis coexist.

Keywords: Granuloma; Immunoglobulin G4-related disease; Lymphadenopathy; Plasma cells.

Figure 1

Perifollicular histiocytic granulomas that formed a wreath around the entire follicle. A: Hematoxylin and eosin stain; B: CD138 immunohistochemical stain highlights the plasma cells; C: Immunoglobulin G (IgG) immunohistochemical stain; D: IgG4 immunohistochemical stain demonstrated that more than 80% of IgG⁺ cells were positive for IgG4.