Ocular and Oral Mucous Membrane Pemphigoid (Cicatricial Pemphigoid)

Abstract

Mucous membrane pemphigoid, a heterogeneous group of autoimmune blistering diseases, affect primarily the mucous membranes. While both oral and ocular mucosae can be affected in a given patient, patients have involvement restricted to oral mucosae tend to have a benign outcome, whereas those with ocular disease commonly face treatment resistance and result in scarring and blindness. Diagnosis requires a direct immunofluorescence microscopy demonstrating a linear deposition of IgG, IgA, or C3 at the epithelial basement membrane. While the target antigens vary, subsets of patients affected exclusively by oral and ocular mucosal diseases have autoantibodies targeting alpha‐6 and beta‐4 integrins, respectively.