Epidermolysis bullosa acquisita (EBA) is an autoimmune blistering skin disease characterized by autoantibodies to type VII collagen. Clinically, a noninflammatory and an inflammatory variant of EBA can be distinguished. Despite major achievements in the understanding of EBA, current therapeutic options are far from optimal. However, with an emerging and more detailed understanding of the events ultimately leading to blister formation in EBA, novel therapeutic options may become available for patients with EBA. Therefore, this article reviews the current understanding of the pathogenesis of EBA and may indicate possible avenues towards a more targeted therapy for EBA and possibly other antibody-mediated autoimmune diseases.