Danescu Sorina, Negrutiu Mircea, Has Cristina
Department of Dermatology, University of Medicine Iuliu Hatieganu Cluj-Napoca, Cluj-Napoca, Romania.
Department of Dermatology, Medical Center University of Freiburg, Freiburg im Breisgau, Germany.
Dermatol Ther (Heidelb). 2024 Aug;14(8):2059-2075. doi: 10.1007/s13555-024-01227-8. Epub 2024 Aug 2.
Epidermolysis bullosa (EB) comprises rare genetic disorders characterized by skin and mucosal membrane blistering induced by mechanical trauma. Molecularly, pathogenic variants affect genes encoding proteins crucial for epidermal-dermal adhesion and stability. Management of severe EB is multidisciplinary, focusing on wound healing support, ensuring that patients thrive, and complication treatment. Despite extensive research over 30 years, novel therapeutic approaches face challenges. Gene therapy and protein therapy struggle with efficacy, while regenerative cell-based therapies show limited effects. Drug repurposing to target various pathogenic mechanisms has gained attention, as has in vivo gene therapy with drugs for dystrophic and junctional EB that were recently approved by the US Food and Drug Administration (FDA) and European Medicines Agency (EMA). However, their high cost limits global accessibility. This review examines therapeutic advancements made over the past 5 years, exploiting a systematic literature review and clinical trial data.
大疱性表皮松解症(EB)是一种罕见的遗传性疾病,其特征是机械创伤引起皮肤和粘膜水疱。在分子层面,致病变异影响编码对表皮-真皮粘附和稳定性至关重要的蛋白质的基因。严重EB的治疗是多学科的,重点是伤口愈合支持、确保患者茁壮成长以及并发症治疗。尽管经过30多年的广泛研究,但新的治疗方法仍面临挑战。基因治疗和蛋白质治疗在疗效方面存在困难,而基于细胞的再生疗法效果有限。针对各种致病机制的药物重新利用已受到关注,美国食品药品监督管理局(FDA)和欧洲药品管理局(EMA)最近批准的用于营养不良性和交界性EB的体内基因治疗药物也是如此。然而,它们的高成本限制了全球可及性。本综述利用系统的文献综述和临床试验数据,考察了过去5年所取得的治疗进展。