Abstract：
Inherited epidermolysis bullosa is a heterogeneous group of hereditary skin diseases characterized by skin (mucosa) fragility, which leads to blistering. Junctional epidermolysis bullosa is associated with mutations in genes expressing proteins of the dermo-epidermal junction. Dupilumab, an antibody that directly targets interleukin (IL)-4 receptor alpha, may be an effective treatment for dystrophic epidermolysis bullosa. We describe a case of junctional epidermolysis bullosa that improved with dupilumab.
摘要：
遗传性大疱性表皮松解症是一组异质性的遗传性皮肤病，其特征是皮肤（粘膜）脆性，导致起泡。连接性大疱性表皮松解症与表达真皮-表皮连接蛋白的基因突变相关。Dupilumab,一种直接靶向白细胞介素（IL）-4受体α的抗体，可能是治疗营养不良性大疱性表皮松解症的有效方法。我们描述了一例使用dupilumab改善的交界性大疱性表皮松解症。
