Department of Dermatology, Kurume University School of Medicine, and Kurume University Institute of Cutaneous Cell Biology, 67 Asahimachi, Kurume, Fukuoka 830-0011, Japan, Department of Maxillofacial Surgery, Aichi-Gakuin University School of Dentistry, Nagoya, Aichi, Japan.
Department of Dermatology, Kurume University School of Medicine, and Kurume University Institute of Cutaneous Cell Biology, 67 Asahimachi, Kurume, Fukuoka 830-0011, Japan.
Eur J Dermatol. 2016 Apr 1;26(2):155-63. doi: 10.1684/ejd.2015.2719.
To confirm that sera from some BP patients reactive exclusively to the BP230 and to study the clinical and immunological characteristics of this condition.
BP patients were divided into three groups: BP reactive only to BP230 (BP230-BP), BP reactive to both BP180 and BP230 (BP180-BP230-BP) and BP reactive only to BP180 (BP180-BP), based on the results of standard ELISAs for BP180 and BP230. Clinical features were statistically analyzed among the three groups. Then, targeted epitopes in each group were studied by immunoblotting and novel ELISAs using three domain-specific BP230 recombinant proteins.
Forty-one, 65 and 47 of 153 BP patients were categorized as BP230-BP, BP180-BP230-BP and BP180-BP, respectively. Clinically, BP230-BP patients showed significantly lower severity, less need of systemic steroids and better responses to various treatments, suggesting that BP230-BP is a milder condition. Immunoblotting and ELISAs of domain-specific BP230 recombinant proteins indicated that, while BP180-BP230-BP sera reacted with all three domains of BP230, BP230-BP sera reacted more frequently with epitopes in the BP230 C-terminal domain.
We propose a new disease entity, named anti-BP230-type BP, in which anti-BP230 antibodies might be pathogenic and react specifically with the BP230 C-terminal domain. While anti-BP230 antibodies in BP180-BP230-BP seem to be produced via intermolecular epitope spreading, anti-BP230 antibodies in BP230-BP are considered to be produced by different mechanisms.
确认某些 BP 患者的血清仅对 BP230 产生反应，并研究这种情况的临床和免疫学特征。
根据 BP180 和 BP230 的标准 ELISA 结果，将 BP 患者分为仅对 BP230 反应的 BP230-BP（BP230-BP）、同时对 BP180 和 BP230 反应的 BP180-BP230-BP（BP180-BP230-BP）和仅对 BP180 反应的 BP180-BP（BP180-BP）三组。对三组患者的临床特征进行统计学分析。然后，通过免疫印迹和使用三种域特异性 BP230 重组蛋白的新型 ELISA 研究每组的靶向表位。
在 153 例 BP 患者中，41、65 和 47 例分别归类为 BP230-BP、BP180-BP230-BP 和 BP180-BP。临床上，BP230-BP 患者的严重程度明显较低，对全身性类固醇的需求较少，对各种治疗的反应更好，这表明 BP230-BP 是一种较轻的疾病。对域特异性 BP230 重组蛋白的免疫印迹和 ELISA 分析表明，虽然 BP180-BP230-BP 血清与 BP230 的三个域均反应，但 BP230-BP 血清更频繁地与 BP230 C 端域的表位反应。
我们提出了一种新的疾病实体，命名为抗 BP230 型 BP，其中抗 BP230 抗体可能具有致病性，并特异性与 BP230 C 端域反应。虽然 BP180-BP230-BP 中的抗 BP230 抗体似乎是通过分子间表位扩展产生的，但 BP230-BP 中的抗 BP230 抗体被认为是通过不同的机制产生的。
