Pemphigoid: Clinical, histologic, immunopathologic, and therapeutic considerations

Autoimmune blistering diseases are generally distinct entities characterize d by relatively consistent clinical, histologic, and immunopathologic findi ngs. These disorders may cause impaired adhesion of epidermis to epidermal basement membrane (eg, the pemphigoid group of disorders [bullous, gestatio nal, and mucous membrane]) or impaired adhesion of epidermal cells to each other (eg, the pemphigus group of disorders). Recent studies have shown tha t these disorders are characterized by autoantibodies that often display pa thogenic (ie, blister-forming) activity in passive transfer models. Interes tingly, the autoantigens targeted by these patients' autoantibodies represe nt important structural proteins that promote cell matrix (eg, pemphigoid) or cell-to-cell (eg, pemphigus) adhesion in skin. Autoimmune blistering dis eases are characterized by substantial morbidity (pruritus, pain, disfigure ment), and in some instances, mortality (secondary to loss of epidermal bar rier function). Treatment with systemic immunosuppressives has reduced morb idity and mortality in patients with these diseases.