Immunophenotypic analysis of the inflammatory infiltrate in ocular cicatricial pemphigoid. Further evidence for a T cell-mediated disease
Pemphigoid, Benign Mucous Membrane
Skin Diseases, Vesiculobullous
Ocular cicatricial pemphigoid (OCP) is characterized by the deposition of immunoglobulin and complement along the conjunctival epithelial basement membrane zone (BMZ). In order to further elucidate the cellular populations of the local inflammatory infiltrates, the authors used a panel of monoclonal antibodies in cryostat tissue sections to delineate T cell subsets, B lymphocytes, dendritic cells, and macrophages in six patients with OCP. In comparison with matched controls of the epibulbar conjunctiva, the authors discovered a threefold increase in T lymphocytes within the epithelium and a 20-fold increase within the substantia propria. In contrast with the normal-standing population of conjunctival T lymphocytes, there were activated interleukin 2 receptor (IL-2R)-positive lymphocytes in both the epithelium and the substantia propria. Macrophages were the second most common cells in the substantia propria, accounting for 12.7% of the mononuclear population--a threefold increase over the normal percentage. B cells and plasma cells, normally absent from epibulbar conjunctiva, were the next most prominent populations, constituting 6.9 and 4.6%, respectively, of all mononuclear cells. Dendritic cells which process antigen locally constituted only 1.2% of the mononuclear cell population, but were increased 25-fold over normal controls. By elaborating cytokines that promote fibroplasia, the T cells in OCP may be effector cells along with macrophages and other inflammatory cells in bringing about scarification of the substantia propria, and may furthermore be responsible for an immunoregulatory defect that allows local B lymphocytes to produce autoantibodies to the BMZ.