Pemphigoid gestationis: maternal sera recognize epitopes restricted to the N-terminal portion of the extracellular domain of BP180 not present on its shed ectodomain.
Pemphigoid gestationis (PG) is an autoimmune subepidermal blistering disease associated with pregnancy and characterized by linear deposition of C3 and, less frequently, of immunoglobulin (Ig)G along the dermal-epidermal junction (DEJ). Complement-fixing circulating IgG autoantibodies to the DEJ in serum of PG patients are mainly directed to the 180-kDa bullous pemphigoid antigen (BP180), a transmembrane hemidesmosomal glycoprotein. BP180 consists of an intracellular N-terminal globular head, a transmembrane region, and a C-terminal ectodomain.