We report the case of a 66-year-old man who presented with a spontaneous blistering involving both skin and mucous membranes, diagnosed as an inflammatory form of epidermolysis bullosa acquisita. During a 5-year course, the condition failed to respond to various therapies. Finally, only ciclosporin, together with a low dose regimen of prednisone, achieved a dramatic improvement with a total clearing within 6 months, persisting 1 1/2 years after discontinuation. Such results prompt us to propose such an association as a primary therapeutic scheme in an otherwise barely tractable disease.