Bullous systemic lupus erythematosus responding to dapsone

A 29-year-old woman with a 4-week history of systemic lupus erythematosus presented acutely with a severe generalized tense vesicular and bullous eruption with involvement of mucosal surfaces. At the time of her initial diagnosis of systemic lupus erythematosus, she had declined treatment, preferring to explore complementary medical therapies. Skin biopsy showed subepidermal blister formation with inflammation at the dermoepidermal junction. Direct immunofluorescence revealed strongly positive linear deposition of IgG and IgM, and positive linear granular deposition of IgA along the basement membrane zone. Electron microscopy showed that the level of the basement membrane split was below the lamina densa. A diagnosis of bullous systemic lupus erythematosus was made and dapsone was commenced, with a dramatic improvement in her skin eruption. The patient again declined further treatment of her systemic disease and sought complementary therapies, and subsequently presented with cerebral involvement.