Sarret Y, Reano A, Nicolas J F, Su H, Thivolet J
Laboratoire de Recherche Dermatologique et Immunologie. INSERUM U. 209, CNRS UA. 601. Hopital E. Herriot, Lyon, France.
Autoimmunity. 1989;2(2):145-53. doi: 10.3109/08916938909019951.
Bullous pemphigoid (BP) and cicatricial pemphigoid (CP) are subepidermal bullous autoimmune diseases which have distinct clinical features but identical immunological status. In order to determine whether these diseases could be dissociated on the basis of qualitative differences in serum antibodies to basement membrane zone (BMZ) antigens, the reactivity of sera from 7 CP and 29 BP patients with proteins extracted from normal human epidermal sheets (containing most of the lamina lucida components) was analysed using immunoblotting and compared to that of 10 normal sera. 20 out of the 29 BP sera contained antibodies recognizing one or several protein(s) of 240, 200, 180 and 165 kD molecular weight (MW). Antibodies in 4 out 7 CP sera specifically reacted with one or two polypeptides of 240 and 120 kD MW. These data confirm the heterogeneity of BP antigens and show the presence in CP of a novel 120 kD MW polypeptide which is found only in CP but not in BP. Taken together these findings demonstrate that in BP and CP, autoantibodies are directed to both common and specific BMZ antigens, their physiopathological significance need to be understood.
大疱性类天疱疮（BP）和瘢痕性类天疱疮（CP）是表皮下大疱性自身免疫性疾病，它们具有不同的临床特征，但免疫状态相同。为了确定这些疾病是否可以根据血清中针对基底膜带（BMZ）抗原的抗体的定性差异来区分，我们使用免疫印迹法分析了7例CP患者和29例BP患者的血清与从正常人表皮片（包含大部分透明层成分）中提取的蛋白质的反应性，并与10例正常血清进行了比较。29例BP血清中有20例含有识别分子量（MW）为240、200、180和165kD的一种或几种蛋白质的抗体。7例CP血清中有4例的抗体与分子量为240和120kD的一种或两种多肽发生特异性反应。这些数据证实了BP抗原的异质性，并显示CP中存在一种仅在CP中发现而在BP中未发现的新的分子量为120kD的多肽。综上所述，这些发现表明，在BP和CP中，自身抗体针对的是共同的和特定的BMZ抗原，其病理生理学意义有待进一步了解。
