Department of Dermatology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.
J Am Acad Dermatol. 2013 Jan;68(1):93-7, 97.e1-2. doi: 10.1016/j.jaad.2012.04.012. Epub 2012 May 26.
Epidermolysis bullosa (EB) is a genetic, heterogeneous, trauma-induced blistering disease. Patients with laminin-332-deficient non-Herlitz junctional EB (JEB-nH) can have impaired wound healing witnessed by persistent, small, deep ulcers on the hands and feet that adversely affect the quality of life.
We sought to present the results of punch grafting in patients with laminin-332-deficient JEB-nH, and to discuss its therapeutic value.
Retrospective analysis of the Dutch EB Registry revealed 4 patients with laminin-332-deficient JEB-nH who were treated with punch grafting. Punch grafting was performed according to protocol, and the patients were followed up.
In the past 10 years we have treated 23 ulcers in 4 patients with JEB-nH using punch grafting without any complications or adverse effects. The ulcers had on average persisted 6 years before treatment. Healing rate after punch grafting was 70% (n = 16), with a mean healing time of 2 months. Thirty percent (n = 7) of the treated ulcers did not completely heal, but did show improvement. The recurrence rate after 3 months was 13% (n = 2), and was a result of renewed blistering.
Limitations of the study are the retrospective design, small number of patients, absence of a control group, and follow-up and ulcer measurement that were not standardized.
Punch grafting can be used as a first-line treatment in small persistent ulcers in patients with JEB-nH. The method is easy, is inexpensive, has little risk of complications, and results in significant healing rates and improvement in quality of life.
大疱性表皮松解症（EB）是一种遗传性、异质性、创伤诱导性的水疱性疾病。层粘连蛋白 332 缺陷型非交界性大疱性表皮松解症（JEB-nH）患者由于手部和足部持续存在小而深的溃疡，伤口愈合能力受损，这会对生活质量产生不利影响。
我们旨在介绍层粘连蛋白 332 缺陷型 JEB-nH 患者接受打孔移植术的结果，并讨论其治疗价值。
对荷兰 EB 注册处进行回顾性分析，发现 4 例层粘连蛋白 332 缺陷型 JEB-nH 患者接受了打孔移植术治疗。根据方案进行打孔移植术，对患者进行随访。
在过去 10 年中，我们使用打孔移植术治疗了 4 例 JEB-nH 患者的 23 处溃疡，没有任何并发症或不良反应。这些溃疡在接受治疗前平均持续了 6 年。打孔移植术后的愈合率为 70%（n=16），平均愈合时间为 2 个月。30%（n=7）的治疗溃疡未完全愈合，但有所改善。3 个月后的复发率为 13%（n=2），这是由于重新出现水疱。
本研究的局限性在于回顾性设计、患者数量少、缺乏对照组以及随访和溃疡测量未标准化。
打孔移植术可作为 JEB-nH 患者小面积持续性溃疡的一线治疗方法。该方法简单、经济、并发症风险低，愈合率高，生活质量显著改善。
