Studies of Cicatricial Pemphigoid Autoantibodies Using Direct Immunoelectron Microscopy and Immunoblot Analysis

作者: Philippe Bernard , Catherine Prost , Véronique Lecerf , Liliane Intrator , Patrick Combemale

DOI: 10.1111/1523-1747.EP12876206

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摘要: We studied 11 consecutive patients with classical cicatricial pemphigoid (CP) using direct immunoelectron microscopy (IEM) and Western immunoblotting analysis. Direct IEM performed in the skin or gingival mucosa revealed all CP that immunoglobulins complement deposits were usually thick discontinuous along dermoepidermal junction, mostly localized on lamina densa occasionally lucida. By IEM, ultra-structural aspect differs from pattern observed bullous (BP) of chronic epidermolysis bullosa acquisita (EBA). Nine by and, these nine, only two had detectable anti-basement membrane zone (BMZ) antibodies indirect immunofluorescence salt-split skin. protein extracts heat-separated epidermis, eight out nine sera specifically reacted bands approximately 230 – 240 kD 180 kD, similar to those recognized BP co-migration experiments. BMZ extracts, none 290-kD major polypeptide EBA antigen. Taken together, results suggest that, CP, target-antigen, as identified immunoblots, is antigen, but an abnormal expression within junction patients, which may part explain scarring course disease.

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