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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.
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. Confc: We called our case as epidermolysis bullosa acquisita with autoantibody to anti-p200 pemphigoid antigen. This is the second case in the literature associated with autoant ibodies to these two antigens.