Systemic lupus erythematosus occurring in a patient with epidermolysis bullosa acquisita.

A dystrophic bullous eruption that met all the criteria for epidermolysis bullosa acquisita (EBA) developed in a 19-year-old woman five years before the onset of clinical and serologic evidence of systemic lupus erythematosus (SLE). Electron microscopic studies of skin lesions both before and after the development of SLE were consistent with the previously reported electron microscopic findings in patients with EBA. Direct immunofluorescence microscopic studies done on bullae before and after the diagnosis of SLE showed linear depositions of immunoglobulin and complement; indirect immunofluorescence microscopic study findings consistently showed no abnormalities. These findings have been noted in other cases of EBA and may implicate autoimmune, immunologic factors in the pathogenesis of the disease process. To our knowledge, the finding of SLE in association with EBA has not been previously reported.