An In Vitro Model of Bullous Pemphigoid - Complement activation and production of complement-derived chemotactic activity is an important functional property of tissue deposited immune complexes. It has been implicated in the pathogenesis of inflammation in a number of diseases associated with immune complex deposits at the basement membrane zone, but its relative importance in these diseases is not well understood due in part to an inability to quantitate the function and determine its relationship to histologic features of skin lesions. In previous studies of one of the diseases, bullous pemphigoid, methods were developed for measuring this function. In preliminary studies, evidence has been obtained that the ability of immune complexes to express this function varies among diseases and that there is a good correlation between this function and histologic features characteristic of immune complex and complement mediated inflammation. In the present study, we plan to measure this function in a variety of diseases, determine its relationship to histologic features and its relationship to the ability of immune complexes to mediate inflammation in organ culture and experimental animals. Studies on Epidermolysis Bullosa Acquisita Antibodies - Epidermolysis bullosa acquisita (EBA) antibodies are anti-basement membrane zone autoantibodies regarded as specific for the disease, EBA. They can be distinguished from other known anti-basement membrane zone autoantibodies by immunofluorescent and immunoultrastructural criteria and by reactivity with 290 and 145 kd proteins extracted from dermis-lamina densa. We have obtained preliminary evidence that EBA-like antibodies are present in patients with bullous eruption of SLE and nonbullous SLE and in an SLE-like murine model of chronic graft vs. host disease in whom autoantibody production is due to a defined H-2 incompatibility. Our preliminary studies also suggest production of EBA autoantibodies in man is closely linked to a specific class II histocompatibility antigen. In this study, we plan to determine if EBA antibodies are present in the circulation and cutaneous immune deposits in SLE, determine if murine models of SLE are associated with EBA antibodies and characterize the class I and II histocompatibility antigen in patients with EBA antibodies.

National Institute of Health (NIH)
National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
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General Medicine A Subcommittee 2 (GMA)
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University of North Carolina Chapel Hill
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Jones, D A; Hunt 3rd, S W; Prisayanh, P S et al. (1995) Immunodominant autoepitopes of type VII collagen are short, paired peptide sequences within the fibronectin type III homology region of the noncollagenous (NC1) domain. J Invest Dermatol 104:231-5
Shimizu, K; Hashimoto, T; Fukuda, T et al. (1995) A Japanese case of the fibrillar type of dermatitis herpetiformis. Dermatology 191:88-92
Gammon, W R; Briggaman, R A (1993) Epidermolysis bullosa acquisita and bullous systemic lupus erythematosus. Diseases of autoimmunity to type VII collagen. Dermatol Clin 11:535-47
Thuong-Nguyen, V; Kadunce, D P; Hendrix, J D et al. (1993) Inhibition of neutrophil adherence to antibody by dapsone: a possible therapeutic mechanism of dapsone in the treatment of IgA dermatoses. J Invest Dermatol 100:349-55
Gammon, W R; Briggaman, R A (1993) Bullous SLE: a phenotypically distinctive but immunologically heterogeneous bullous disorder. J Invest Dermatol 100:28S-34S
Jenison, M; Fine, J D; Gammon, W R et al. (1993) Normal molecular weight of type VII collagen produced by recessive dystrophic epidermolysis bullosa keratinocytes. J Invest Dermatol 100:93-6
Dmochowski, M; Hashimoto, T; Bhogal, B S et al. (1993) Immunoblotting studies of linear IgA disease. J Dermatol Sci 6:194-200
Gammon, W R; Murrell, D F; Jenison, M W et al. (1993) Autoantibodies to type VII collagen recognize epitopes in a fibronectin-like region of the noncollagenous (NC1) domain. J Invest Dermatol 100:618-22
Gammon, W R; Abernethy, M L; Padilla, K M et al. (1992) Noncollagenous (NC1) domain of collagen VII resembles multidomain adhesion proteins involved in tissue-specific organization of extracellular matrix. J Invest Dermatol 99:691-6
Domloge-Hultsch, N; Benson, P; Gammon, W R et al. (1992) A bullous skin disease patient with autoantibodies against separate epitopes in 1 mol/L sodium chloride split skin. Arch Dermatol 128:1096-101

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