UNUSUAL, CUTANEOUS, CICATRICIAL PEMPHIGOID ASSOCIATED WITH ANTIBODIESOF THE IGG AND IGA CLASS REACTING WITH THE BP180 NC16A DOMAIN

We present a case of a 70-year-old female patient with localized cutan eous cicatricial pemphigoid [CP] of 11 years duration confined to the periumbilical area and not involving mucous membranes. Because of the unusual clinical presentation, this case was not recognized for a long time. The diagnosis was confirmed by an immunopathologic study which disclosed IgG and C3 along the basement membrane zone [BMZ] and high t iter [640] of circulating BMZ antibodies. In salt-split skin, antibodi es reacted with the roof of the bulla: strongly with IgG, and much mor e weakly with IgA. Importantly, in immunoblot both IgG and IgA antibod ies reacted with 180 fusion protein, but not with the human epidermal extract. This is the first report on the reactivity of IgA BMZ antibod ies with the BP 180 NC16a domain in cutaneous CP.