背景:遗传性大疱性表皮松解症(EB)是一种以皮肤脆性和独特口腔特征为特征的遗传性疾病。
目的:提供(a)对患有每种类型遗传性EB的人的口腔表现的完整回顾,(b)目前管理EB患者口腔保健的最佳做法,(c)针对隐性营养不良性EB(RDEB)患者的基于种植牙的口腔修复的当前最佳实践,以及(d)目前管理局部麻醉的最佳做法,镇静原则,和全身麻醉的儿童和成人EB接受牙科治疗。
方法:系统文献检索,小组讨论包括来自世界各地不同中心的临床专家和患者代表,外部审查,和指南驾驶。
结果:本文分为五章:(i)口腔保健专业人员关于EB的一般信息,(ii)关于EB的口头表现的系统文献综述,(iii)符合EB-临床实践指南的儿童和成人的口腔保健和牙科治疗;(iv)符合RDEB临床实践指南的患者的牙科植入物,和(v)成人和接受牙科治疗的EB儿童的镇静和麻醉-临床实践指南。每一章都提供了有关牙科实践中不同临床程序的管理建议。强调患者与临床医生伙伴关系的重要性,对生活质量的影响,以及后续任命的重要性。提供了关于使用非粘附性伤口护理产品和润肤剂以减少患者护理期间的摩擦的指导。
结论:遗传性EB的口腔软组织和硬组织表现具有与每种亚型相关的独特受累模式。单独了解每个亚型将有助于专业人员计划长期治疗方法。
BACKGROUND: Inherited epidermolysis bullosa (EB) is a genetic disorder characterized by skin fragility and unique oral features.
OBJECTIVE: To provide (a) a complete review of the oral manifestations in those living with each type of inherited EB, (b) the current best practices for managing oral health care of people living with EB, (c) the current best practices on dental implant-based oral rehabilitation for patients with recessive dystrophic EB (RDEB), and (d) the current best practice for managing local anesthesia, principles of sedation, and general anesthesia for children and adults with EB undergoing dental treatment.
METHODS: Systematic literature search, panel discussion including clinical experts and patient representatives from different centers around the world, external review, and
guideline piloting.
RESULTS: This article has been divided into five chapters: (i) general information on EB for the oral health care professional, (ii) systematic literature review on the oral manifestations of EB, (iii) oral health care and dental treatment for children and adults living with EB-clinical practice
guidelines, (iv) dental implants in patients with RDEB-clinical practice
guidelines, and (v) sedation and anesthesia for adults and children with EB undergoing dental treatment-clinical practice
guidelines. Each chapter provides recommendations on the management of the different clinical procedures within dental practice, highlighting the importance of patient-clinician partnership, impact on quality of life, and the importance of follow-up appointments. Guidance on the use on nonadhesive wound care products and emollients to reduce friction during patient care is provided.
CONCLUSIONS: Oral soft and hard tissue manifestations of inherited EB have unique patterns of involvement associated with each subtype of the condition. Understanding each subtype individually will help the professionals plan long-term treatment approaches.