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一种罕见的断肢症与表皮松解性鱼鳞病的关联,在Z形皮瓣修复失败后通过全厚皮片移植成功治愈。

A novel association of pseudoainhum and epidermolytic ichthyosis, successfully treated with full thickness skin graft after failed z-plasty repair.

作者信息

Simkin David, Ho Jonathan D, Simkin Daren J, Tomany Kevin

机构信息

5New York University School of Medicine, Department of Internal Medicine, New York. daren.

出版信息

Dermatol Online J. 2018 Jan 15;24(1):13030/qt1ph217qf.

Abstract

Pseudoainhum is a rare constriction band variant thatmay progress to spontaneous digital strangulationand auto-amputation. Although its association withpalmoplantar keratodermas is well established, ithas not been reported in conjunction with classicepidermolytic ichthyosis. We describe the first suchcase in a 25-year-old woman who presented witha painful constricting band of the fifth toe. We alsodescribe her treatment course, which consisted ofa failed z-plasty, the traditional therapeutic optionfor acute pseudoainhum, and report the success ofsubsequent full thickness skin graft, suggesting thebenefit of this procedure as a therapeutic alternativefor patients with pseudoainhum.

摘要

假断肢是一种罕见的缩窄带变体,可能进展为自发性手指绞窄和自截。尽管其与掌跖角化病的关联已得到充分证实,但尚未见与经典表皮松解性鱼鳞病相关的报道。我们描述了首例这样的病例,患者为一名25岁女性,其第五趾出现疼痛性缩窄带。我们还描述了她的治疗过程,包括一次失败的Z成形术(急性假断肢的传统治疗选择),并报告了随后全厚皮片移植的成功,提示该手术作为假断肢患者的一种治疗替代方法的益处。

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