Autoantibodies in a subgroup of patients with linear IgA disease react with the NC16A domain of BP180

Molecular Mapping of the Major Epitopes of BP180 Recognized by Herpes Gestationis Autoantibodies

“…Both BP and HG autoantibodies react with NC16A segments contained in NC16A2 (MCW-1), A2.5 and A3, and, in addition, some BP sera also reacted with NC16A1. Lichen planus pemphigoides autoantibodies react primarily with a site in the C-terminal portion of NC16A4 (25), and a major autoantibodyreactive region associated with cicatricial pemphigoid is located near the C-terminus of the BP180 protein (19,27). Thus, at the fine specificity level, HG autoantibodies are highly similar to BP autoantibodies, but exhibit differences from those produced by cicatricial pemphigoid and lichen planus pemphigoides patients-differences that might account for differences observed at the clinical level.…”

“…Lane 5, rabbit anti-NC16A (1:1000). 290 subepithelial blistering disorders, which include, in addition to HG, BP (17), cicatricial pemphigoid (19), linear IgA disease (25), and lichen planus pemphigoides (26). These disorders are characterized by subepithelial (mostly subepidermal) inflammatory blisters and the presence of autoantibodies directed against the BP180 antigen.…”