Histopathologic and immunohistopathologic features of subretinal fibrosis and uveitis syndrome.

We studied histopathologic and immunohistopathologic features of an eye from a patient who had hypopigmented choroidal lesions with subsequent development of progressive subretinal fibrotic tissue associated with minimal signs of vitreal inflammation. A 24-year-old woman had a rapid and severe onset of the disease, which led to blindness within a few months despite treatment with corticosteroids and cyclophosphamide. Histopathologic studies disclosed a marked gliotic retina and thick subretinal fibrotic tissue as well as a granulomatous lymphocytic infiltration in the choroid. Electron microscopy demonstrated that the subretinal tissue was derived from retinal pigment epithelial cells. The immunoperoxidase staining showed similar proportions of T and B lymphocytes, indicating a relative increase of B cells. There was a predominance of the helper/inducer T lymphocyte subset in the infiltrating area. The Müller cells in the retina expressed class II antigens of the major histocompatibility complex.