Response of Intractable Skin Ulcers in Recessive Dystrophic Epidermolysis Bullosa Patients to an Allogeneic Cultured Dermal Substitute
Response of Intractable Skin Ulcers in Recessive Dystrophic Epidermolysis Bullosa Patients to an Allogeneic Cultured Dermal Substitute
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DOI：
10.2340/00015555-0776
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发表时间：
2010-01-01
影响因子：
3.6
通讯作者：
Shimizu, Hiroshi
中科院分区：
文献类型：
--
作者：
Natsuga, Ken;Sawamura, Daisuke;Shimizu, Hiroshi
隐性营养不良性大疱性表皮松解症 (RDEB) 是一种遗传性皮肤病，由编码 VII 型胶原蛋白 (COL7) 的 COL7A1 基因突变引起。 RDEB 患者的皮肤溃疡有时愈合缓慢。我们在这里描述了两名全身性 RDEB 患者的顽固性皮肤溃疡对同种异体培养真皮替代品 (CDS) 治疗的治疗反应。治疗后 3-4 周，两名患者的皮肤溃疡均出现上皮化。免疫组织化学研究表明，与对照皮肤相比，COL7 表达水平仍然降低，并且移植处理区域和未处理区域之间没有显着差异。电子显微镜显示两个样本的致密层下方存在异常的锚定原纤维。总之，CDS 是治疗 RDEB 患者顽固性皮肤溃疡的一种有前景的方法，尽管它似乎不会增加 COL7 的表达。
Recessive dystrophic epidermolysis bullosa (RDEB) is an inherited skin disorder caused by mutations in the COL7A1 gene, which encodes collagen VII (COL7). Skin ulcers in RDEB patients are sometimes slow to heal. We describe here the therapeutic response of intractable skin ulcers in two patients with generalized RDEB to treatment with an allogeneic cultured dermal substitute (CDS). Skin ulcers in both patients epithelialized by 3-4 weeks after this treatment. Immunohistochemical studies demonstrated that the COL7 expression level remained reduced with respect to the control skin and that it did not differ significantly between graft-treated and untreated areas. Electron microscopy showed aberrant anchoring fibrils beneath the lamina densa of both specimens. In conclusion, CDS is a promising modality for treatment of intractable skin ulcers in patients with RDEB, even though it does not appear to increase COL7 expression.