Published online Jul 16, 2015. doi: 10.12998/wjcc.v3.i7.650
Peer-review started: January 28, 2015
First decision: March 20, 2015
Revised: April 13, 2015
Accepted: May 16, 2015
Article in press: May 18, 2015
Published online: July 16, 2015
Processing time: 182 Days and 12.3 Hours
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.
Core tip: We report a case of a 38-year-old man who presented with worsening left eye pain and multiple lymphadenopathy. An axillary lymph node resection showed increased eosinophils and plasma cells (at least 80% of immunoglobulin (Ig)G+ plasma cells were positive for IgG4 and the presence of multifocal, perifollicular histiocytic granulomas, which formed a wreath around the entire follicles. An extensive workup was negative for lymphoma, fungal, or mycobacterial infection. The findings were suggestive of IgG4-related sclerosing disease-associated lymphadenopathy. Thus, IgG4-related lymphadenopathy should be listed in the differential diagnoses of benign reactive lymph nodes, especially when perifollicular granuloma and plasmacytosis coexist.