Bullous systemic lupus erythematosus: detection of antibodies against noncollagenous domain of type VII collagen.

A 9-year-old girl with systemic lupus erythematosus developed bullous eruption. Histopathologic examination showed subepidermal blistering and immunoglobulins (IgG, IgM, IgA) and complement components (C1q, C3) deposited linearly at the basement membrane zone. IgG in this patient's serum bound to the dermal side of 1 mol/L sodium chloride-split normal human skin, and a 290 kd protein was identified in the dermal extract. The patient's serum recognized various epitopes on the noncollagenous domain as demonstrated by the use of fusion proteins of type VII collagen. Resolution of the bullous eruption occurred after treatment with dapsone.