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Contrasting Outcomes of Surgical versus Natural History in Dystrophic Epidermolysis Bullosa at 26 Months

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Mendeley Data2026-04-18 收录
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Mendeley Supplementary figure 1A: Preoperative presentation. Bilateral, symmetric end-stage "mitten" or "club" hand deformity is evident. Complete pseudosyndactyly with digital fusion renders the hands functionally useless. Mendeley Supplementary figure 1B:Postoperative state at 26-month follow-up after digital separation of the right hand. This image provides a direct, long-term comparison. The surgically treated right hand maintains visible interdigital separation despite underlying disease. Preserved in a functional, bandaged posture, it enables essential activities of daily living, such as self-feeding. In stark contrast, the contralateral, non-operated left hand demonstrates the relentless natural history of severe DEB, exhibiting marked progression of pseudosyndactyly, near-complete obliteration of the interdigital spaces, and worsening contracture.

Mendeley补充图1A:术前表现。可见双侧对称性终末期“手套状”或“棍棒状”手部畸形。完全性假性并指(pseudosyndactyly)伴手指融合,致使双手完全丧失功能。 Mendeley补充图1B:右手手指分离术后26个月随访的术后状态。本图像可提供直接的长期对照。尽管存在基础疾病,经手术治疗的右手仍可见清晰的指间隙分离。该手维持于绷带固定的功能位,可完成进食等必要的日常生活活动。与之形成鲜明对比的是未接受手术的对侧左手,其呈现出重症营养不良性大疱性表皮松解症(Dystrophic Epidermolysis Bullosa, DEB)难以遏制的自然病程进展:假性并指显著加重,指间隙近乎完全闭塞,挛缩程度进一步恶化。
创建时间:
2026-04-06
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