Background:Some cases of a subepidermal blistering disease associated with autoantibodies to more than two antigens have been reported.Observation:A 52-year-old Japanese woman had pruritic blisters on almost the whole...Background:Some cases of a subepidermal blistering disease associated with autoantibodies to more than two antigens have been reported.Observation:A 52-year-old Japanese woman had pruritic blisters on almost the whole body as well as erosive lesions in the oral cavity and esophagus.A histological finding was subepidermal bullae.Direct immunofluorescence(IF)revealed a linear deposition of IgG,IgM and C3 at the epidermal basement membrane zone(BMZ).Indirect IF using human skin split by 1 M NaCl as a substrate showed IgG antibody reactive with the dermal side.By immunoblot analysis using normal human dermal extract,the 200-kDa and 290-kDa bands were detected.Indirect IF did not show any anti-BMZ antibody activity,when using the skin of the patient with recessive dystrophic epidermolysis bullosa as a substrate.Conclusion:We regarded our case as epidermolysis bullosa acquisita with autoantibody to anti-p200 pemphigoid antigen.This is the second case in the literature associated with autoantibodies to these two antigens.展开更多
文摘Background:Some cases of a subepidermal blistering disease associated with autoantibodies to more than two antigens have been reported.Observation:A 52-year-old Japanese woman had pruritic blisters on almost the whole body as well as erosive lesions in the oral cavity and esophagus.A histological finding was subepidermal bullae.Direct immunofluorescence(IF)revealed a linear deposition of IgG,IgM and C3 at the epidermal basement membrane zone(BMZ).Indirect IF using human skin split by 1 M NaCl as a substrate showed IgG antibody reactive with the dermal side.By immunoblot analysis using normal human dermal extract,the 200-kDa and 290-kDa bands were detected.Indirect IF did not show any anti-BMZ antibody activity,when using the skin of the patient with recessive dystrophic epidermolysis bullosa as a substrate.Conclusion:We regarded our case as epidermolysis bullosa acquisita with autoantibody to anti-p200 pemphigoid antigen.This is the second case in the literature associated with autoantibodies to these two antigens.