Autoimmune Skin Conditions: Autoimmune Blistering Disease

There are many types of autoimmune blistering skin disease. Two of the most common are bullous pemphigoid and pemphigus vulgaris. Bullous pemphigoid is characterized by tense bullae created by a subepidermal split resulting from autoantibodies targeted at the hemidesmosomes at the dermal-epidermal junction. Bullous pemphigoid typically occurs in elderly people and often can be drug-induced. Pemphigus vulgaris is characterized by flaccid bullae because of an intraepithelial split triggered by autoantibodies targeting desmosomes. Diagnosis can be made for both conditions by physical examination, biopsy for routine histology, biopsy for direct immunofluorescence, and serologic studies. Both bullous pemphigoid and pemphigus vulgaris are associated with significant morbidity and mortality and diminished quality of life, making early recognition and diagnosis paramount. Management proceeds in a stepwise approach using potent topical corticosteroids along with immunosuppressant drugs. Rituximab recently has been shown to be the drug of choice for most people with pemphigus vulgaris.