Steroid-sensitive nephrotic syndrome associated with Kimura disease

We report an 11-year-old Japanese boy with Kimura disease and associated ne phrotic syndrome. Before the diagnosis of Kimura disease was established, t he patient had three episodes of swelling on the left cheek with subsequent nephrotic syndrome. Steroids were effective for both conditions. However, both conditions recurred within months of discontinuation of steroids. For the fourth episode of swelling on the left cheek, cyclosporine (CsA) was us ed. The subcutaneous tumor responded to CsA and disappeared within a few da ys. There has been no subsequent relapse of the nephrotic syndrome to date.