Adhesive stripping to remove epidermis in junctional epidermolysis bullosa for revertant cell therapy

Adhesive stripping to remove epidermis in junctional epidermolysis bullosa for revertant cell therapy
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DOI:
10.1111/j.1365-2133.2009.09118.x
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发表时间:
2009-08-01
影响因子:
10.3
通讯作者:
Jonkman, M. F.
Jonkman, M. F.
中科院分区:
医学1区
文献类型:
--
作者:
Gostynski, A.;Deviaene, F. C. L.;Jonkman, M. F.

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背景用转导的自体角质形成细胞的上皮片层替换大疱性表皮病(epidermelicbullosa,EB)中的突变皮肤是离体基因治疗的基本外科步骤。这同样适用于回复突变细胞疗法,其中使用回复突变自体角质形成细胞的上皮片层。回复突变细胞可以发现在补丁的正常皮肤的交界性EB(JEB)的患者由于回复突变嵌合体引起的体内reversions.ObjectivesTo开发一种技术的胶带剥离作为一种方法,表皮去除准备回复突变嵌合体JEB.MethodsWe患者的回复突变体细胞治疗的受体位点与嵌合型XVII胶原蛋白缺乏非Herlitz JEB。皮肤活检取自手腕上的回复突变皮肤。在3 T3-J2饲养层上进行移植物生产,得到两个6 x 7 cm移植物。开发了一种创新的方法,该方法使用JEB皮肤的最小阻力的病理平面来制备受体部位。将聚丙烯酸酯橡皮膏置于皮肤上,然后与表皮一起撕下。皮肤在透明板水平分离,留下裸露致密板的无血伤口床。移植是成功的;受体部位愈合无疤痕。然而,起泡可能会被挑起。由于回复突变细胞在graft.ConclusionsWe的百分比低,功能修复没有实现,得出结论,粘合剥离是一个简单,有效,几乎无痛的程序,去除表皮细胞治疗EB。
BackgroundReplacing mutant skin in epidermolysis bullosa (EB) by epithelial sheets of transduced autologous keratinocytes is the essential surgical step of ex vivo gene therapy. The same applies for revertant cell therapy in which epithelial sheets of revertant autologous keratinocytes are used. Revertant cells can be found in patches of normal skin in patients with junctional EB (JEB) due to revertant mosaicism caused by in vivo reversions.ObjectivesTo develop a technique of adhesive tape stripping as a method for epidermis removal to prepare the acceptor site for revertant cell therapy in a patient with revertant mosaic JEB.MethodsWe performed revertant cell therapy on a patient with mosaic type XVII collagen-deficient non-Herlitz JEB. Skin biopsies were taken from revertant skin on the wrist. Graft production took place on a 3T3-J2 feeder layer resulting in two 6 x 7 cm grafts. An innovative method that uses the pathological plane of least resistance of JEB skin was developed to prepare the acceptor site. A polyacrylate adhesive plaster was placed on the skin and then pulled off with the epidermis.ResultsThe epidermis was easily removed with the plaster. The skin separated at the level of the lamina lucida, leaving a bloodless wound bed of naked lamina densa. Transplantation was successful; the acceptor site healed without scarring. However, blistering could be provoked. The functional repair was not achieved due to the low percentage of revertant cells in the graft.ConclusionsWe conclude that adhesive stripping is a simple, effective and almost painless procedure for removing epidermis for ex vivo cell therapy in EB.