Epidermolysis bullosa acquisita: diagnosis by fluorescence overlay antigen mapping and clinical response to high-dose intravenous immunoglobulin

Epidermolysis bullosa acquisita (EBA) is a chronic, subepidermal blistering disease characterized by the presence of autoantibodies to type VII collagen, located below the lamina densa of the basement membrane zone (BMZ). There is a large clinical and histological overlap between EBA and other subepidermal autoimmune bullous diseases, therefore, complex immunological techniques are required to make an accurate diagnosis. Therapy of EBA is also a difficult issue. Most patients do not respond to several common immunosuppressive agents. We describe a patient who has shown a good response to high-dose intravenous immunoglobulin therapy.