NONSCARRING INFLAMMATORY EPIDERMOLYSIS-BULLOSA ACQUISITA WITH ESOPHAGEAL INVOLVEMENT AND LINEAR IGG DEPOSITS

A 24-year-old woman with autoimmune thrombocytopenia and hypothyroidis m had an inflammatory bullous eruption in the mouth, face, and trunk t hat left no milia or scars after healing. Histologic examination revea led a subepidermal bulla and a neutrophil infiltration. Direct immunof luorescence examination showed deposition of IgG and C3 in the basemen t membrane zone (BMZ). Indirect immunofluorescence examination with 1M sodium chloride-split skin showed IgG binding to the dermal side. Imm unoblot analysis demonstrated IgG autoantibodies reacting with 290 kD dermal protein. We diagnosed this as epidermolysis bullosa acquisita ( EBA) with a nonscarring inflammatory feature. Treatment with oral daps one, 75 mg, and prednisolone, 20 mg, cleared the eruption. Reduction o f the prednisolone dosage was associated with multiple erosions in the esophagus. Direct immunofluorescence examination revealed linear depo sition of IgG in the esophageal BMZ. To our knowledge, this is the fir st report of EBA with esophageal involvement and deposition of IgG in the BMZ of the esophagus.