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.
Highlights
The autoimmune bullous diseases are caused by an immune response to proteins of the desmosomes or the basement membrane zone (BMZ).[1]
Subepithelial bullae, Linear immunoglobulin and complement deposition along BMZ detected by DFA or immunoperoxidase assay, circulating anti‐BM autoantibodies
Mucous membrane pemphigoid (MMP, formerly called ocular cicatricial pemphigoid) is a chronic vesiculobullous disease of the mucous membranes that manifests on the eyes primarily through progressive scarring of the conjunctiva
Summary
The autoimmune bullous diseases are caused by an immune response to proteins of the desmosomes or the basement membrane zone (BMZ).[1]. Quick Response Code: Website: www.jovr.org pemphigoid, pemphigus vulgaris, paraneoplastic pemphigus, linear immunoglobulin A disease, lichen planus, pemphigus foliaceus, dermatitis herpetiformis, and epidermolysis bullosa acquisita [Table 1]. These autoimmune bullous diseases can be differentiated on the basis of characteristic patterns of mucocutaneous involvement and the specific tissue components targeted by the autoantibodies. Ocular pemphigus vulgaris exhibits intraepithelial activity, whereas the autoimmune activity in linear immunoglobulin A disease, mucous membrane pemphigoid, and epidermolysis bullosa
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