Lupus Eritematoso Sistémico Ampolloso: dramática respuesta a la terapia con dapsona

Bullous systemic lupus erythematosus (BSLE) is an unusual finding in systemic lupus erythematosus (SLE). Other bullous disorders such as bullous pemphigoid, epirdermolysis bullosa acquisita, linear IgA bullous dermatosis and dermatitis herpetiformis has also been reported in association with SLE. We described a patient who developed severe bullous lesions 14 days after high-dose systemic glucocorticoids and cyclophosphide therapies were initiated for severe SLE manifestations (nephritis and alveolar hemorrhage). A diagnosis of bullous SLE was made. Therapy with dapsone resulted in a marked clinical improvement of the bullous eruption within 48 hours. This article also discusses the epidemiology, clinical, histopathologic and immunopathologic features, differential diagnosis and the treatment of BSLE.