Bullous systemic lupus erythematosus with lupus nephritis: a rare case of a subepidermal bullous disorder in a child.

Bullous systemic lupus erythematosus (BSLE) is a rare subset of systemic lupus erythematosus (SLE) with bullous lesions in a case fulfilling the American Rheumatism Association (Atlanta) criteria, histologically characterized by a neutrophil-predominant infiltrate in the upper dermis with immunoglobulin (IgG, IgA, IgM) and C3 deposition at the basement membrane zone (BMZ). It often is associated with autoimmunity to type VII collagen (NC1 [noncollagenous domain 1] domain), although occasionally other antigens such as laminin 5, laminin 6, and BP230 (bullous pemphigoid antigen) have been described. Bullous systemic lupus erythematosus is extremely rare in children. We report here a case of a 9-year-old girl with BSLE as an initial presentation with lupus nephritis class III, a rare occurrence at such a young age. Despite the rarity, we suggest that BSLE should be considered in the differential diagnosis of subepidermal bullous diseases in children in view of associated potentially serious systemic manifestations.