Oral health care pathways for patients with epidermolysis bullosa: A position statement from the European reference network for rare skin diseases

S Krämer; A L Hillebrecht; K Bekes; K Bücher; V Clark; H Haririan; J Jakowski; C Joseph; N Meißner; J Monteiro; S Porter; R Schilke; S Veliz; V Verhaeghe; A Vinereanu; M C Bolling; A Diem; J E Mellerio; C Bodemer; C Has

doi:10.1111/jdv.20498

Oral health care pathways for patients with epidermolysis bullosa: A position statement from the European reference network for rare skin diseases

J Eur Acad Dermatol Venereol. 2024 Dec 13. doi: 10.1111/jdv.20498. Online ahead of print.

Authors

S Krämer^{1

2

3}, A L Hillebrecht⁴, K Bekes⁵, K Bücher⁶, V Clark⁷, H Haririan⁸, J Jakowski⁹, C Joseph^{2

10}, N Meißner¹¹, J Monteiro¹², S Porter¹³, R Schilke¹⁴, S Veliz¹⁵, V Verhaeghe^{2

16}, A Vinereanu^{2

17}, M C Bolling^{2

18}, A Diem^{2

19}, J E Mellerio²⁰, C Bodemer^{2

21}, C Has^{1

2}

Affiliations

¹ Department of Dermatology and Venereology, Medical Center - University of Freiburg, Freiburg, Germany.
² ERN-Skin (European Network for Rare Skin Disease).
³ Special Care Dentistry Unit, Facultad de Odontología, Universidad de Chile, Santiago, Chile.
⁴ Department of Prosthetic Dentistry, Centre for Dental Medicine, Medical Centre - University of Freiburg, Freiburg, Germany.
⁵ Department of Paediatric Dentistry, University Clinic of Dentistry, Medical University of Vienna, Vienna, Austria.
⁶ Section for Paediatric Dentistry, Department of Conservative Dentistry and Periodontology, University Hospital, Ludwig-Maximilians-University, Munich, Germany.
⁷ Birmingham Children's Hospital, Birmingham, UK.
⁸ Department of Periodontology, Dental Clinic, Faculty of Medicine, Sigmund Freud University, Vienna, Austria.
⁹ Department of Oral Surgery and Dental Emergency Care, Faculty of Health, Witten/Herdecke University, Witten, Germany.
¹⁰ Department of Paediatric Dentistry, CHU de Nice, Faculty of Dentistry, Côte d'Azur University, Nice, France.
¹¹ Private Practice, Salzburg/Berlin, Germany.
¹² Department of Paediatric Dentistry, Sheffield Teaching Hospitals, Sheffield, UK.
¹³ Institute Director and Professor of Oral Medicine, UCL Eastman Dental Institute, University College London, London, UK.
¹⁴ Department of Conservative Dentistry, Periodontology and Preventive Dentistry, Hannover Medical School, Hannover, Germany.
¹⁵ Faculty of Medicine and Dentistry, University of Santiago de Compostela, Santiago de Compostela, Spain.
¹⁶ Department of Pediatric Dentistry and Special Care, University Hospitals Leuven, Leuven, Belgium.
¹⁷ Pedodontics Department, Carol Davila University of Medicine and Pharmacy Bucharest, Bucharest, Romania.
¹⁸ Department of Dermatology, University Medical Centre Groningen, UMCG Expertise Centre for Blistering Diseases, University of Groningen, Groningen, The Netherlands.
¹⁹ EB House Austria, Department of Dermatology and Allergology, University Hospital of the Paracelsus Medical University, Salzburg, Austria.
²⁰ St John's Institute of Dermatology, Guy's and St Thomas' NHS Foundation Trust, London, UK.
²¹ Department of Dermatology, Necker Enfants-Malades Hospital, Paris Cité University, APHP, Paris, France.

PMID: 39673192
DOI: 10.1111/jdv.20498

Abstract

Background: 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.

Objective: To guide clinicians caring for patients with different types of EB to seek care from different dental services.

Methods: 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.

Results: 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).

Conclusions: 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.