Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan.
Acta Derm Venereol. 2010 Mar;90(2):165-9. doi: 10.2340/00015555-0776.
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.
隐性营养不良型大疱性表皮松解症（RDEB）是一种遗传性皮肤疾病，由 COL7A1 基因突变引起，该基因编码 VII 型胶原（COL7）。RDEB 患者的皮肤溃疡有时愈合缓慢。我们在此描述了两名全身性 RDEB 患者对同种异体培养的真皮替代物（CDS）治疗的难治性皮肤溃疡的治疗反应。在这种治疗后 3-4 周，两名患者的皮肤溃疡均开始上皮化。免疫组织化学研究表明，与对照皮肤相比，COL7 的表达水平仍然降低，且在移植治疗区和未治疗区之间没有显著差异。电子显微镜显示两个标本的板层致密下方存在异常锚定纤维。总之，CDS 是治疗 RDEB 患者难治性皮肤溃疡的一种有前途的方法，尽管它似乎不会增加 COL7 的表达。
