Cicatricial pemphigoid: Immunopathogenesis and treatment

Cicatricial pemphigoid (CP) is a chronic, autoimmune, subepidermal blistering disease of mucous membranes and skin that has a tendency to scar and result in tissue destruction. Recent studies demonstrate that CP is a heterogeneous disease in which patients can resemble each other clinically, histologically, and immunopathologically, but have autoantibodies that target different autoantigens in the epidermal basement membrane. Accordingly CP is now considered to be a disease phenotype rather than a single nosologic entity. CP can be associated with substantial morbidity, and in rare instances, mortality. Currently the management of patients with CP is grounded in clinical experience rather than the results of large randomized trials. This article discusses recent advances in the understanding of this disorder's pathophysiology and treatment.