Krämer S, Hillebrecht A L, Bekes K, Bücher K, Clark V, Haririan H, Jakowski J, Joseph C, Meißner N, Monteiro J, Porter S, Schilke R, Veliz S, Verhaeghe V, Vinereanu A, Bolling M C, Diem A, Mellerio J E, Bodemer C, Has C
Department of Dermatology and Venereology, Medical Center - University of Freiburg, Freiburg, Germany.
ERN-Skin (European Network for Rare Skin Disease).
J Eur Acad Dermatol Venereol. 2025 Jun;39(6):1080-1090. doi: 10.1111/jdv.20498. Epub 2024 Dec 13.
Inherited epidermolysis bullosa (EB) comprises a group of genetic disorders characterized by skin fragility and unique oral features. It requires interdisciplinary care from several health professionals, including oral health teams. Modern dentistry encompasses a wide range of therapeutic options performed by specialists from different fields.
To guide clinicians caring for patients with different types of EB to seek care from different dental services.
Dental treatment needs for patients with EB were identified based on a systematic literature review. A panel of experts was consulted and invited to provide additional information through an open-ended question over 7 months. A Delphi study was applied over two rounds to the resulting pathways design. The threshold of consensus was set a priori at 75%. Patients' representatives revised the final document.
The panel (n = 17) agreed on a total of 55 recommendations divided into six groups according to the severity of oral compromise in EB (52 recommendations were agreed on in round 1, and three were agreed on in round 2).
Dental care pathways are presented for each type of EB. Specific considerations are discussed according to clinical features, including age of first referral, frequency of follow-up appointments, and list of dental specialties involved in the care of patients with EB.
遗传性大疱性表皮松解症（EB）是一组以皮肤脆弱和独特口腔特征为特点的遗传性疾病。它需要包括口腔健康团队在内的多名健康专业人员提供跨学科护理。现代牙科涵盖了不同领域专家所采用的广泛治疗选择。
指导照顾不同类型EB患者的临床医生向不同牙科服务机构寻求护理。
基于系统的文献综述确定EB患者的牙科治疗需求。咨询了一个专家小组，并邀请他们在7个月内通过一个开放式问题提供更多信息。对所得的途径设计进行了两轮德尔菲研究。事先将共识阈值设定为75%。患者代表对最终文件进行了修订。
该小组（n = 17）共达成了55项建议，根据EB口腔损害的严重程度分为六组（第一轮达成52项建议，第二轮达成3项建议）。
针对每种类型的EB都提出了牙科护理途径。根据临床特征讨论了具体注意事项，包括首次转诊年龄、随访预约频率以及参与EB患者护理的牙科专业列表。
