Peripheral corneal ulcer associated with benign hypergammaglobulinemic purpura

We describe a 56-year-old woman with benign hypergammaglobulinemic purpura in whom scleritis, acute stromal keratitis and peripheral corneal furrowing developed. Initial therapy with topical antibiotics and steroids failed to control the scleritis and keratitis. Subsequent treatment with plasmapheresis led to a decrease in circulating immune complex and serum immunoglobulin levels, with resolution of the ocular inflammation. After 8 years of follow-up the serum IgG and IgM levels were again elevated; however, the corneal disease remained quiescent. Plasmapheresis may have a role in the therapy of this and other immune-complex-mediated ocular diseases.