Three Cases of Bullous Pemphigoid with only Anti-BP230 Antibody

Abstract

Bullous pemphigoid (BP) is an autoimmune blistering disease in which autoantibodies to BP antigens (BP 230 and/or BP180) are thought to be pathogenic. We report herein three cases of BP showing only anti-BP230 antibodies. All 3 cases (a 73-year-old male, a 54-year-old female, and a 59-year-old female) had generalized erythema, blisters and erosions, which immediately disappeared when treated with oral corticosteroids and/or double filtration plasmapheresis. In all 3 cases, immunoblot analysis failed to detect anti-BP180 antibodies, although antibodies to the C-terminus of BP230 were detected. The detailed roles and functions of anti-BP230 Ab in BP are still obscure. It remains necessary to clarify the pathogenicity of anti-BP230 antibodies by accumulating information about the relationship between clinical presentations and antibody profiles in more cases with BP.