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Abstract:  We report a case of linear immunoglobulin A bullous disease in a 9-year-old boy who presented with rapidly progressive severe disease and could not tolerate dapsone because of high liver enzymes within a week after a low dose of dapsone in association with an underlying fatty liver. He showed remarkable improvement with intravenous immunoglobulins used as monotherapy, with a rapid clearance and a sustained remission after stopping the treatment.