Abstract
Pemphigoid gestationis is unique as an organ-specific autoimmune disease closely associated with pregnancy. It belongs to the pemphigoid group of disorders characterized by subepidermal blister formation and autoantibodies targeting cutaneous basement membrane-zone antigens. The fine specificity of autoantibodies in pemphigoid gestationis is highly restricted and confined to the NC16A domain of collagen XVII (BP180). Data from an animal model of pemphigoid are unraveling the process from anti-NC16A antibodies to blister formation. However, the initiating trigger of the autoimmune response remains enigmatic. This review discusses the current understanding of the pathogenesis of pemphigoid gestationis, the role of immunogenetics, immune privilege of the fetoplacental unit and adaptations of the maternal immune system, as well as the treatment and potential impact of pemphigoid gestationis on the fetus.