Autoimmune Blistering Diseases

Autoimmune Bullous Disease Introduction

The bullous skin diseases, although uncommon, may have substantial morbidity. Coupled with the clinical presentation, these autoimmune disorders are characterized by their immunopathology and histopathology. Pathogenic autoantibodies bind to antigens with adhesion functions within the epidermis or dermis. The level of split within the skin or mucous membranes dictates the specific morphology. The target antigens are components of intercellular desmosomes or the adhesion complex, which is the basement membrane zone (BMZ) functional unit. However, target antigens and specific epitopes may be found with more than one disorder. The overlap between blistering disorders can make a definitive diagnosis challenging. Even when the specific target antigen is known, the exact pathogenesis for many bullous diseases is not fully understood.

Immunofluorescence (IF), along with the histopathologic features, has become the mainstay of diagnosis and has helped...