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

我们对哥伦比亚南美El Bagre地区的落叶型天疱疮患者进行了大规模流行病学研究。首先，从房屋、饮食、经济条件等方面收集了发病的要点。同时，我们从许多患者身上采集了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采集了血液样本。通过临床表现和调查研究，发现这些患者具有特异的免疫学特征。也就是说，通过免疫荧光检测，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，我们通过各种方法检查了患者血清。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。用ELISA法检测Dsg1和Dsg3，几乎所有患者血清都有Dsg1抗体。此外，通过使用人表皮提取物的相同免疫印迹，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BP 230反应。为了证实这些结果，我们进行免疫印迹使用重组蛋白的envoplakin和periplakin。在这项研究中，许多患者血清与periplakin的重组蛋白反应，但不与envoplakin的重组蛋白。用BP230重组蛋白进行免疫印迹，几乎所有血清均为阴性

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