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遗传性大疱性表皮松解症的皮肤外表现及并发症:第一部分。上皮相关组织。

Extracutaneous manifestations and complications of inherited epidermolysis bullosa: part I. Epithelial associated tissues.

作者信息

Fine Jo-David, Mellerio Jemima E

机构信息

The National Epidermolysis Bullosa Registry, and Department of Medicine (Dermatology), Vanderbilt University School of Medicine, Nashville, Tennessee, USA.

出版信息

J Am Acad Dermatol. 2009 Sep;61(3):367-84; quiz 385-6. doi: 10.1016/j.jaad.2009.03.052.

Abstract

Based upon case reports and small case series, it has been known for many years that some types and subtypes of inherited epidermolysis bullosa (EB) may be at risk for developing one or more extracutaneous complications. Many of these are associated with considerable morbidity; some may result in death. Only over the past few years have there been data generated from large, well characterized cohorts. However, these data, to date, have been published almost exclusively in the nondermatologic literature. Our objective is to provide dermatologists with a comprehensive review of each major extracutaneous complication with a summary of the pertinent literature and recommendations for evaluation and optimal management. Part I highlights epithelial associated tissues, and part II addresses other organs. Based on these reviews, the readership should gain a greater understanding of the types of complications that may occur, when they are most likely to develop, and the range of medical and surgical interventions that are currently available. It should also be possible for the reader to develop surveillance strategies based on an understanding of the published evidence-based data. The breadth and range of severity of complications that arise in some EB types and subtypes within the external eye, ear, nose, upper airway, and gastrointestinal and genitourinary tracts suggest that optimal management must be multidisciplinary. Given the unique knowledge that dermatologists have of this disease, we believe that the care of the EB patient should be under the direction of his or her dermatologist, who can best assist in timely referrals to those specialists who are most experienced in the care of specific extracutaneous problems.

摘要

基于病例报告和小病例系列研究,多年来人们已经知道,某些类型和亚型的遗传性大疱性表皮松解症(EB)可能有发生一种或多种皮肤外并发症的风险。其中许多并发症会导致相当高的发病率;有些可能会导致死亡。直到过去几年,才从大型、特征明确的队列研究中获得相关数据。然而,迄今为止,这些数据几乎只发表在非皮肤病学文献中。我们的目标是为皮肤科医生提供一份关于每种主要皮肤外并发症的全面综述,总结相关文献,并给出评估和最佳管理建议。第一部分重点介绍与上皮相关的组织,第二部分讨论其他器官。基于这些综述,读者应该能更好地理解可能发生的并发症类型、最可能出现的时间,以及目前可用的医疗和外科干预措施的范围。读者还应该能够根据对已发表的循证数据的理解制定监测策略。某些EB类型和亚型在外眼、耳、鼻、上呼吸道以及胃肠道和泌尿生殖道中出现的并发症的广度和严重程度范围表明,最佳管理必须是多学科的。鉴于皮肤科医生对这种疾病有独特的了解,我们认为EB患者的护理应由其皮肤科医生指导,皮肤科医生能够最好地协助及时转诊到那些在处理特定皮肤外问题方面最有经验的专家。

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