| Research Abstract |
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
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