Pemphigus. Skin failure mediated by autoantibodies.

Pemphigus is a group of diseases in which autoantibodies mediate a loss of cell-to-cell adhesion, resulting in blister formation and ultimate failure of the barrier function of the skin. These autoantibodies are directed against complexes that contain polypeptides found in adhering junctions. Exactly how these antibodies mediate loss of cell adhesion, whether through secondary mechanisms such as release of proteases, direct interference of junction formation, or both, remains an area of active and exciting investigation.