Follicular dendritic cell sarcoma with paraneoplatic pemphigus: Rare case and a brief review of literature

Abstract

Paraneoplastic pemphigus (PNP) is often a fatal autoimmune bullous disease characterized by severe stomatitis, polymorphous skin eruptions, and underlying neoplasms. We describe a patient with PNP associated with follicular dendritic cell sarcoma (FDCS), a rare neoplasm originating from follicular dendritic cells, which are non-lymphoid, non-phagocytic accessory cells of the lymphoid system and play an integral role in regulation of the germinal center reaction and present antigens to B-cells. The presence of rich vascularity around the tumor and few hyalanized vascular follicles found in histopathological examination gives the clue that the tumor might have developed from Castleman's disease (CD). As for the mechanisms by which CD induces PNP, it has been proposed that autoantibodies secreted from the Castleman's tumor play pivotal role. This hypothesis seems to be supported by the present case, in which CD may have triggered both the FDCS and the PNP.