Basic studies for the pathogenesis and diagnosis of the autoimmune bullous diseases

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There are a number of autoimmune blistering diseases. We have investigated various aspects in these diseases under a collaborative basis with many investigators in foreign institutes. Nishikawa T,Amagai M and Hashimoto T have been abroad in a past year, exchanged various materials for experiments and established a number of collaborative projects. Particularly, the antigens for various types of pemphiqus and various antigens for IgA antibodies in sera of either dermatitis herpetiformis and linear IgA dermatosis have been studied extensively with Dr.John R.Stanley and Dr.John J.Zone, respectively. With Dr.Stanley in NIH,U.S., We investigated on the pemphiqus vulgaris antigen and the pemphigus foliaceus antigen (desmoglein). Using their cDNA and baculovirus expression system, we produced recombinant proteins for both the pemphigus vulgaris antigen and the pemphigus foliaceus antigen (desmoglein) with proper conformation. Absorption of the pathogenic antibodies in the sera of both pemphig … More us vulgaris and pemphigus foliaceus patients was confirmed by the experiments that the treatment of the sera with these recombinant baculoproteins inhibit completely blister formation in newborn mouse animal experiment. This indicates the possibility of new therapy of antigen specific affinity column using these recombinant proteins. With Dr.Zone in Utah University, U.S., we studied on diseases in which IgA antibodies are involved. We analyzed a Japanese case with rare fibrillar type of dermatitis herpetiformis, in particular on various IgA autoantibodies. Furthermore, we investigated on the autoantigens for linear IgA bullous dermatosis, and found that most common antigen seems to be a 97 kD protein which is present in the epidermal side of the 1M NaCl split skin. Very recently, we have also found that the 290 kD epidermolysis bullosa acquisita antigen is recognized by some linear IgA bullous dermatosis sera. These collaborative researches should further provide us with many new insights for the progress in both diagnosis and therapy in various autoimmune skin diseases in the future. Less