Kimura's disease associated with membranous nephropathy with IgG4 and phospholipase A2 receptor-positive staining of the glomerular basement membrane.

Kimura's disease is a granulomatous disease of unknown origin that develops in the dermis, subcutaneous tissue and lymph nodes. Kimura's disease is frequently complicated by nephropathy, particularly membranous nephropathy (MN). It has recently been suggested that glomerular immunoglobulin (IgG)4 deposition may play a role in the pathogenesis of idiopathic MN. These IgG4 antibodies are thought to react with antigens, primarily the phospholipase A2 receptor (PLA2R) expressed on the podocyte cell membrane. We herein report a case of Kimura's disease with MN in which a renal biopsy specimen revealed positive staining for anti-IgG4 and anti-PLA2R antibodies.