Clinical Information

Bullous pemphigoid (BP) is a chronic pruritic blistering disorder found mainly in aged persons, characterized by the development of tense blisters over an erythematous or urticarial base. IgG anti-basement membrane zone antibodies are found in the serum of patients, and linear IgG and complement component 3 sediment is found on the basement membrane zone of the lesion. Several well characterized variants exist including localized, mucous membrane predominant and pemphigoid gestationis, also referred to as herpes gestationis.

Target antigens of the autoantibodies in BP patient serum are BP230 and BP180, also called BPAG1 and BPAG2. Molecular weight of these antigens is 230 kDa and 180 kDa, respectively. BP180 is thought to be the direct target of the autoantibody because of its location along the basement membranes, and the autoantibody against BP230 is thought to be secondarily produced.