A 24-year-old woman with autoimmune thrombocytopenia and hypothyroidism had an inflammatory bullous eruption in the mouth, face, and trunk that left no milia or scars after healing. Histologic examination revealed a subepidermal bulla and a neutrophil infiltration. Direct immunofluorescence examination showed deposition of IgG and C3 in the basement membrane zone (BMZ). Indirect immunofluorescence examination with 1M sodium chloride-split skin showed IgG binding to the dermal side. Immunoblot analysis demonstrated IgG autoantibodies reacting with 290 kD dermal protein. We diagnosed this as epidermolysis bullosa acquisita (EBA) with a nonscarfing inflammatory feature. Treatment with oral dapsone, 75 mg, and prednisolone, 20 mg, cleared the eruption. Reduction of the prednisolone dosage was associated with multiple erosions in the esophagus. Direct immunofluorescence examination revealed linear deposition of IgG in the esophageal BMZ. To our knowledge, this is the first report of EBA with esophageal involvement and deposition of IgG in the BMZ of the esophagus.