Abstract
Several cases have been reported of patients with immunemediated subepidermal blistering disorders whose autoantibodies react to antigens present on both the dermal and epidermal side of 1 M NaCl-split skin. In this report, we identify, localize, and characterize the basement membrane zone antigen corresponding to the dermal staining in a patient whose serum stains both the dermal and epidermal side of 1 M NaCl-split skin. This patient's serum contains autoantibodies directed against a 105-kilodalton(kDa) dermal antigen and the 230-kDa epidermal (bullous pemphigoid) antigen. This novel 105-kDa protein was previously identified as the sole antigen in another patient with a unique bullous disease whose autoantibodies were directed against only the dermal side of 1 M NaCl-split skin. This 105-kDa antigen was identical by one- and two-dimensional immunoblot analysis in these two patients. By immunoblot analysis, autoantibodies from our patient labeled a 105-kDa protein within various extracts of human skin basement membrane. Immunoblot analyses using epitope-selected autoantibodies directed against the 105-kDa protein demonstrated that this antigen is independent and distinct from other known basement membrane antigens. The 105-kDa antigen is an extracellular matrix component of the basement membrane, which is synthesized and secreted by both keratinocytes and fibroblasts. Identical electrophoretic migration of cellular and secreted forms of the protein suggested there is no major post-translational modification of the protein. Immunomapping of normal human skin fractured through the dermal-epidermal junction by incubation in 1 M NaCl or by suction blistering demonstrated that the location of the 105-kDa antigen within the basement membrane zone is between the bullous pemphigoid antigens and two other lamina lucida components, laminin and nicein. These data demonstrate clearly that a subepidermal autoimmune bullous disease may have autoantibodies directed against two distinct components of the dermal-epidermal junction.
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