Immunoglobulin A (IgA) is the most common subtype of antibodies in mucosal surfaces. In most of autoimmune bullous diseases, however, immunoglobulin G (IgG) is the main pathogenic antibody that plays a role through complementation. The IgA antibody for epidermal connection protein can be found in the sera of some patients with blistering skin disease. Of these patients, some have the IgA antibody in their sera, while others have IgG and IgA antibodies. IgA-related autoimmune bullous diseases are less common in clinical practice. In the past, these diseases were not fully understood and their classifications were confusing. Recently, some progress has been made in the study of these diseases.