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从内到外:隐性营养不良型大疱性表皮松解症的再生医学。

Inside out: regenerative medicine for recessive dystrophic epidermolysis bullosa.

机构信息

Division of Blood and Marrow Transplantation, Department of Pediatrics, University of Minnesota, Minneapolis, Minnesota.

Stem Cell Institute, University of Minnesota, Minneapolis, Minnesota.

出版信息

Pediatr Res. 2018 Jan;83(1-2):318-324. doi: 10.1038/pr.2017.244. Epub 2017 Nov 1.

Abstract

Epidermolysis bullosa is classified as a genodermatosis, an inherited genetic skin disorder that results in severe, chronic skin blistering with painful and life-threatening complications. Although there is currently no cure for epidermolysis bullosa, concurrent advances in gene and stem cell therapies are converging toward combinatorial therapies that hold the promise of clinically meaningful and lifelong improvement. Recent studies using hematopoietic stem cells and mesenchymal stromal/stem cells to treat epidermolysis bullosa have demonstrated the potential for sustained, effective management of the most severe cases. Furthermore, advances in the use of gene therapy and gene-editing techniques, coupled with the development of induced pluripotent stem cells from patients with epidermolysis bullosa, allow for autologous therapies derived from a renewable population of cells that are patient-specific. Here we describe emerging treatments for epidermolysis bullosa and other genodermatoses, along with a discussion of their benefits and limitations as effective therapies.

摘要

大疱性表皮松解症被归类为一种遗传性皮肤病,是一种遗传性皮肤疾病,会导致严重的慢性皮肤水疱,伴有疼痛和危及生命的并发症。尽管目前尚无治疗大疱性表皮松解症的方法,但基因和干细胞疗法的同步进展正在朝着联合疗法汇聚,这些疗法有望带来具有临床意义和终身改善的效果。最近使用造血干细胞和间充质基质/干细胞治疗大疱性表皮松解症的研究表明,有可能对最严重的病例进行持续有效的治疗。此外,基因治疗和基因编辑技术的进步,以及从大疱性表皮松解症患者中诱导多能干细胞的发展,使得可以从可再生的患者特异性细胞群体中获得自体疗法。在这里,我们描述了新兴的大疱性表皮松解症和其他遗传性皮肤病的治疗方法,并讨论了它们作为有效治疗方法的益处和局限性。

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