Immunological and epidemiological studies on the endemic pemphigus foliaceus seen in developing areas of South America Columbia

我们对南美洲哥伦比亚埃尔巴格雷地区的地方性落叶型天疱疮患者进行了大规模的流行病学研究。首先，我们收集了房屋、食物、经济条件等发病的要点。同时，我们还采集了许多患者的血液样本和皮肤样本。作为对照，我们还从同一地区的患者亲属和志愿者那里获取了血液样本。通过临床特征和调查，我们发现这些患者具有特定的免疫学特征。那是 ;通过对皮肤样本进行免疫荧光检查，我们发现 IgG 沉积在角质形成细胞表面，免疫球蛋白和补体成分沉积在基底膜区。此外，我们还通过各种方法检查了患者血清。通过对人表皮提取物进行免疫印迹，我们发现大多数患者血清与 160 kDa 桥粒芯糖蛋白 1 (Dsg1) 发生反应。通过ELISA检测Dsg1和Dsg3，发现几乎所有患者血清均含有Dsg1抗体。此外，通过使用人表皮提取物进行相同的免疫印迹，我们发现大多数患者血清与已知的副肿瘤性天疱疮抗原 envoplakin 和 periplakin 以及大疱性类天疱疮的自身抗原 BP230 发生反应。为了证实这些结果，我们使用 envoplakin 和 periplakin 的重组蛋白进行了免疫印迹。在这项研究中，许多患者血清与重组蛋白 periplakin 发生反应，但不与重组蛋白 envoplakin 发生反应。通过使用 BP230 重组蛋白进行免疫印迹，几乎所有血清的 BP230 均为阴性

We have performed a large epidemiological studies on the endemic pemphigus foliaceus patients seen in El Bagre areas, South America, Columbia. First of all, we collected the important points for the pathogenesis in houses, foods, economic conditions. At the same time, we collected both blood samples and skin samples from many patients. As controls, we also obtain blood samples from the relatives of the patients and volunteers in the same areas. By clinical features and investigations, we found that these patients had specific immunological features. That is ; byimmunofluorescence for the skin samples, we found IgG deposition to the keratinocyte cell surfaces, as well as deposition of immunoglobulins and complement components to the basement membrane zone. In addition, we examined the patient sera by various methods. By immunoblotting of human epidermal extracts, we found that the most patient sera react with the 160 kDa desmoglein 1 (Dsg1). By ELISA for Dsg1 and Dsg3, almost all the patient sera were found to have antibodies to Dsg1. Furthermore, by the same immunoblotting using human epidermal extracts, we found that the most patient sera reacted with envoplakin and periplakin, known paraneoplastic pemphigus antigens, as well as BP230, the autoantigen for bullous pemphigoid. To confirm these results, we performed immunoblotting using recombinant proteins of envoplakin and periplakin. In this study, many patient sera reacted with recombinant proteins of periplakin, but not with recombinant proteins of envoplakin. By immunoblotting using recombinant proteins of BP230, almost all the sera were negative for BP230