The clinical efficacy and safety of anti-IgE therapy in recessive dystrophic epidermolysis bullosa,Clinical Genetics

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The clinical efficacy and safety of anti-IgE therapy in recessive dystrophic epidermolysis bullosa
Clinical Genetics ( IF 2.9 ) Pub Date : 2021-09-08 , DOI: 10.1111/cge.14062
Fuying Chen _{1,

2} , Yifeng Guo _{1,

2} , Kaili Zhou _{1,

2} , Dan Deng _{1,

2} , Wanbo Yue _{1,

2} , Weiqin Yang ₁ , Beibei Zhang ₁ , Yue Li _{1,

2} , Jianying Liang _{1,

2} , Ming Li _{1,

2} , Zhirong Yao _{1,

2}

Affiliation

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).

中文翻译：

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

隐性营养不良性大疱性表皮松解症 (RDEB) 的治疗仍然具有挑战性。此前曾在几名 RDEB 患者中报告过 IgE 水平升高。在这项前瞻性、单中心、开放干预研究中，12 名严重瘙痒患者中有 11 名出现 IgE 水平升高，IgE 水平升高的患者每 4 周接受一次抗 IgE 治疗，持续至少三个周期。与基线相比，10 名 RDEB 患者具有良好的临床结果，伤口愈合加快，伯明翰（大疱性表皮松解症）EB 严重程度评分降低 15%，受影响的体表面积减少 23.3%，皮肤炎症改善，以及VII 型胶原沉积增加 13.1 倍。所有患者对抗IgE治疗均具有良好的耐受性。此外，具有较高 IgE 水平的患者往往具有较高的疾病严重程度和更有利的临床结果。我们的报告还提出了 IgE 在与 RDEB 相关的炎症性疾病的发病机制中的潜在作用。(ChiCTR1900021437)。

更新日期：2021-09-08

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