AUTOIMMUNE SUBEPITHELIAL BLISTERING DISEASES WITH OCULAR INVOLVEMENT

Abstract

Autoimmune bullous diseases are a group of blistering diseases characterized by in vivo bound and circulating autoantibodies directed against components of epithelia. They can be divided histologically into two main groups based on the location of the cleavage plane in the epithelia—intraepithelial for the pemphigus group and subepithelial for the group of subepithelial blistering diseases. These two groups of diseases can also be distinguished by the types of molecules that serve as target antigens. Autoantibodies associated with the pemphigus group react with protein constituents of the desmosome, a cell structure that functions in cell-cell adhesion, whereas the autoimmune response in the subepithelial group is directed against components of the basement membrane zone (BMZ). Both groups of bullous diseases are composed of multiple disease entities that are distinguishable at the clinical, histological, and immunological levels. This article focuses on ocular manifestations of autoimmune subepithelial blistering diseases. The diseases that fall into this category are listed in Table 1. They include cicatricial pemphigoid (CP), the prototypic disease with the most severe ocular involvement, epidermolysis bullosa acquisita (EBA), and linear IgA disease (LAD). This article describes the clinical, histological, and immunopathological findings, with an emphasis on ocular involvement. Pathogenesis and treatment are also discussed. Pemphigus vulgaris and paraneoplastic pemphigus, which are defined by an intraepithelial cleavage, and bullous pemphigoid (BP) and dermatitis herpetiformis, which are rarely associated with ocular lesions, are not covered.