Department of Basic Medical Science, The Medical College of Xiamen University, Xiamen, China.
Priority Area Asthma & Allergy, Research Center Borstel, Airway Research Center North (ARCN), Member of the German Center for Lung Research Deutsches Zentrum für Lungenforschung (DZL), Borstel, Germany.
Front Immunol. 2021 Oct 12;12:750160. doi: 10.3389/fimmu.2021.750160. eCollection 2021.
Epidermolysis bullosa acquisita (EBA) is an autoimmune blistering disorder characterized and caused by autoantibodies against type VII collagen (COL7). Although it has been noticed that EBA in both patients and mice is associated with an increased scratching, it is not clear whether and how the scratching contributes to disease manifestation. Hence, we here aimed to validate this clinical observation and also to investigate the potential contribution of increased scratching in EBA pathogenesis in mice. Longitudinal assessment of scratching behavior revealed an increased frequency of scratching as early as 12 hours after injection of anti-COL7 IgG into the skin of mice. Subsequently, scratching events became even more frequent in mice. In contrast, mice injected with a control antibody showed an unaltered scratching behavior throughout the observation period. Based on these observations, we hypothesized that mechanical irritation may promote the induction of inflammation in experimental EBA. To challenge this assumption, the local anesthetic dyclonine hydrochloride was topically applied before injection of anti-COL7 IgG. Dyclonine hydrochloride reduced the scratching events and impaired clinical disease manifestation. In therapeutic experimental settings, i.e. administration of the local anesthetic 24 hours after injection of anti-COL7 IgG, dyclonine hydrochloride only inhibited the scratching behavior, but had no significant effect on clinical disease development. In addition, eosinophils were detected in the skin before the injection of anti-COL7 IgG and significantly increased 48 hours after the antibody injection. Collectively, our results suggest that scratching behavior contributes to the initiation phase of disease manifestation in experimental EBA.
获得性大疱性表皮松解症(EBA)是一种自身免疫性水疱性疾病,其特征和病因是针对 VII 型胶原(COL7)的自身抗体。虽然已经注意到 EBA 患者和小鼠均存在搔抓增加,但尚不清楚搔抓是否以及如何导致疾病表现。因此,我们旨在验证这一临床观察结果,并研究搔抓增加在 EBA 发病机制中的潜在作用。对搔抓行为的纵向评估显示,在将抗 COL7 IgG 注入小鼠皮肤后 12 小时,搔抓的频率就已经增加。随后,搔抓事件在小鼠中变得更加频繁。相比之下,注射对照抗体的小鼠在整个观察期间的搔抓行为没有改变。基于这些观察结果,我们假设机械刺激可能促进实验性 EBA 中的炎症诱导。为了验证这一假设,我们在注射抗 COL7 IgG 之前局部应用局部麻醉剂盐酸达克罗宁。盐酸达克罗宁可减少搔抓事件并损害临床疾病表现。在治疗性实验设置中,即在注射抗 COL7 IgG 后 24 小时给予局部麻醉剂,盐酸达克罗宁仅抑制搔抓行为,但对临床疾病发展没有显著影响。此外,在注射抗 COL7 IgG 之前就已经在皮肤中检测到嗜酸性粒细胞,并且在抗体注射后 48 小时显著增加。综上所述,我们的结果表明搔抓行为可能参与了实验性 EBA 的发病起始阶段。