Onodera Y, Shimizu H, Hashimoto T, Ishiko A, Ebihara T, Tanaka M, Nishikawa T
Department of Dermatology, Keio University School of Medicine, Tokyo, Japan.
J Invest Dermatol. 1994 May;102(5):686-90. doi: 10.1111/1523-1747.ep12374252.
The purpose of this study is to clarify a relationship between bullous pemphigoid autoantibodies against two major BP antigens, 230 kilodalton (kD) (BPAG1) and 180 kD (BPAG2) and their binding sites, using immunoblot analysis and indirect immunofluorescence on salt-split skin. Of the 135 sera obtained from patients in whom bullous pemphigoid had previously been diagnosed by clinical and immunopathologic criteria, all 52 sera recognizing only BPAG1 stained only the epidermal side of split skin (epidermal pattern). Of 24 sera recognizing only BPAG2, 20 showed the epidermal pattern and four stained both the epidermal and dermal sides (combined pattern). Of 42 recognizing both BPAG1 and BPAG2, 35 showed the epidermal pattern and seven showed the combined pattern. Of 17 that reacted with neither antigen, nine showed the epidermal pattern, four showed the combined pattern, and four stained only the dermal side (dermal pattern). Two of the four cases that showed a dermal pattern were retrospectively identified as epidermolysis bullosa acquisita. Immunoelectron microscopy confirmed that a serum with combined pattern bound to both intracellular and extracellular sites of hemidesmosomes. Our results suggest that autoantibodies that react solely with BPAG1 bind exclusively to the epidermal side of salt-split skin and never show either a combined or a dermal pattern, and that most antibodies against BPAG2 bind to the epidermal side. The combined pattern suggests the presence of autoantibodies against the extracellular epitopes of BPAG2 that are separated from the epidermis during the salt-splitting process.
本研究的目的是通过对盐裂皮肤进行免疫印迹分析和间接免疫荧光，阐明针对两种主要大疱性类天疱疮自身抗体（230千道尔顿（kD）（BPAG1）和180 kD（BPAG2））及其结合位点之间的关系。从先前根据临床和免疫病理学标准诊断为大疱性类天疱疮的患者中获得135份血清，所有仅识别BPAG1的52份血清仅染盐裂皮肤的表皮侧（表皮型）。在仅识别BPAG2的24份血清中，20份显示表皮型，4份染表皮和真皮两侧（联合型）。在同时识别BPAG1和BPAG2的42份血清中，35份显示表皮型，7份显示联合型。在与两种抗原均无反应的17份血清中，9份显示表皮型，4份显示联合型，4份仅染真皮侧（真皮型）。显示真皮型的4例病例中有2例经回顾性鉴定为获得性大疱性表皮松解症。免疫电子显微镜证实，具有联合型的血清与半桥粒的细胞内和细胞外位点均结合。我们的结果表明，仅与BPAG1反应的自身抗体仅与盐裂皮肤的表皮侧结合，从不显示联合型或真皮型，并且大多数针对BPAG2的抗体与表皮侧结合。联合型表明存在针对BPAG2细胞外表位的自身抗体，这些表位在盐裂过程中与表皮分离。
