The clinical efficacy and safety of anti-IgE therapy in recessive dystrophic epidermolysis bullosa
The clinical efficacy and safety of anti-IgE therapy in recessive dystrophic epidermolysis bullosa
复制标题
抗IgE治疗隐性营养不良性大疱性表皮松解症的临床疗效和安全性
DOI：
10.1111/cge.14062
复制
发表时间：
2021-09-15
影响因子：
3.5
通讯作者：
Yao, Zhirong
中科院分区：
文献类型：
--
作者：
Chen, Fuying;Guo, Yifeng;Yao, Zhirong
隐性营养不良大疱性表皮松解症（RDEB）的治疗仍然具有挑战性。以前曾报道过几例RDEB患者的IgE水平升高。在这项前瞻性、单中心、开放干预研究中，12例严重瘙痒患者中有11例出现IgE水平升高，IgE水平升高的患者每4周接受至少3个周期的抗IgE治疗。与基线相比，10例RDEB患者具有良好的临床结果，伤口愈合增强，伯明翰（大疱性表皮松解症）EB严重程度评分降低15%，患处体表面积减少23.3%，皮肤炎症改善，VII型胶原沉积增加13.1倍。所有患者对抗ige治疗均有良好的耐受性。此外，IgE水平较高的患者往往有更高的疾病严重程度和更有利的临床结果。我们的报告也提示了IgE在与RDEB相关的炎症发病机制中的潜在作用。(ChiCTR1900021437)。
The treatment of recessive dystrophic epidermolysis bullosa (RDEB) remains challenging. Elevated IgE levels have previously been reported in several RDEB patients. In this prospective, single-centre, open intervention study, elevated IgE levels were seen in 11 out of 12 patients with intense pruritus, and the patients with elevated IgE levels received anti-IgE therapy every 4 weeks for at least three cycles. Compared with the baseline, 10 patients with RDEB had good clinical outcomes with enhanced wound healing, a reduction in Birmingham (epidermolysis bullosa) EB severity score by 15%, a reduction in affected body surface area by 23.3%, amelioration of skin inflammation, and an increase in type VII collagen deposition by 13.1-fold. All the patients had a good tolerance to anti-IgE therapy. Furthermore, patients with higher IgE levels tended to have higher disease severity and more favorable clinical outcomes. Our report also suggested the potential role of IgE in the pathogenesis of inflammatory conditions associated with RDEB. (ChiCTR1900021437).