Abstract
Autoimmune bullous diseases with ocular involvement consist of a group of systemic entities that are characterized by formation of autoantibodies against the proteins of the epithelial basement membrane zone of the conjunctiva. Mostly, the elderly are affected by these diseases. The characteristic patterns of mucocutaneous involvement and the specific tissue components targeted by these autoantibodies are differentiating features of these diseases. Ocular pemphigus vulgaris exhibits intraepithelial activity, whereas the autoimmune activity in linear immunoglobulin A disease, mucous membrane pemphigoid, and epidermolysis bullosa acquisita occurs at a subepithelial location. Given the increased risk for blindness with delays in diagnosis and management, early detection of ocular manifestations in these diseases is vital. The precise diagnosis of these autoimmune blistering diseases, which is essential for proper treatment, is based on clinical, histological, and immunological evaluation. Management usually includes anti-inflammatory and immunosuppressive medications. Inappropriate treatment results in high morbidity and even potential mortality.