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

我们对在南美洲哥伦比亚省巴格雷地区发现的地方性叶状天疱疮患者进行了大规模流行病学研究。首先，我们收集了房屋、食物、经济条件对发病的重要影响。同时，我们采集了许多患者的血样和皮肤样本。作为对照，我们还从患者亲属和同一地区的志愿者那里采集了血液样本。通过临床表现和调查，我们发现这些患者具有特定的免疫学特征。也就是说，通过对皮肤样本的免疫荧光，我们发现免疫球蛋白和补体成分沉积在角质形成细胞表面，以及免疫球蛋白和补体成分沉积在基底膜区域。此外，我们还对患者血清进行了各种方法的检测。通过对人表皮提取物的免疫印迹，我们发现大多数患者血清与160 kDa桥粒芯糖蛋白1(DSG1)反应。通过对DSG1和Dsg3的ELISA检测，几乎所有的患者血清都有DSG1的抗体。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，我们使用包膜蛋白和包膜蛋白的重组蛋白进行了免疫印迹。在本研究中，许多患者血清与Periplakin重组蛋白反应，而不与Envoplakin重组蛋白反应。用BP230重组蛋白进行免疫印迹，几乎所有血清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