Bullous systemic lupus erythematosus (BSLE) is a rare bullous dermatosis in patients with systemic lupus erythematosus. It is characterized by clinical and histologic features, resembling either bullous pemphigoid or dermatitis herpetiformis, and a heterogeneous immunologic profile, characterized by autoimmunity to components of type VII collagen, much like epidermolysis bullosa acquisita. As understanding of the pathology of this interesting dermatologic condition has evolved, so too have criteria and profiling of BSLE. The distinct clinical, histologic, and immunologic features of BSLE represent a unique bullous disease phenotype.