Sporadic diffuse corneal endotheliitis

We studied two patients with acute diffuse corneal endotheliitis who had no family history of the disease. Endothelial specular photographs taken during and after an attack of endotheliitis demonstrate the deposition of inflammatory material on edematous endothelial cells, with areas of focal endothelial cell loss that resolved with treatment. These cases are distinctly different than the previously described "idiopathic" primary endotheliitis entities, which have included focal or sectoral areas of corneal edema, corneal edema in association with a migrating rejection line, or diffuse edema occurring as a dominantly inherited condition. No definitive causative agents have been established. It is likely that the disease results from an immune response to autoantigens or antigens from an unidentified viral infection. The pathogenesis may be similar to that of corneal homograft rejection.