Susanne Krämer1, James Lucas2, Francisca Gamboa1, Miguel Peñarrocha Diago3, David Peñarrocha Oltra3, Marcelo Guzmán-Letelier4,5, Sanchit Paul6, Gustavo Molina7,8, Lorena Sepúlveda1, Ignacio Araya1,9, Rubén Soto1, Carolina Arriagada1, Anne W Lucky10,11, Jemima E Mellerio12, Roger Cornwall10, Fatimah Alsayer13, Reinhard Schilke14, Mark Adam Antal15, Fernanda Castrillón1, Camila Paredes1, Maria Concepción Serrano16, Victoria Clark17. 1. Facultad de Odontología, Universidad de Chile, Santiago, Chile. 2. Dental Department, Royal Children's Hospital, Melbourne, Australia. 3. Stomatology Department, Faculty of Medicine and Dentistry, University of Valencia, Spain. 4. Hospital Base Valdivia, Valdivia, Chile. 5. Facultad de Odontologia, Universidad San Sebastián, Valdivia, Chile. 6. Nurturing Healthy Smiles, Greater Noida, India. 7. Universidad Nacional de Córdoba, Argentina. 8. Universidad Católica de Córdoba, Argentina. 9. Hospital Santiago Oriente, Maxillofacial Surgery Unit, Chile. 10. Cincinnati Children's Epidermolysis Bullosa Center, Cincinnati Children's Hospital, Cincinnati, Ohio, USA. 11. The University of Cincinnati College of Medicine, Cincinnati, Ohio, USA. 12. St John's Institute of Dermatology, Guy's and St Thomas' NHS Foundation Trust, London, UK. 13. Royal National ENT and Eastman Dental Hospitals, University College London Hospitals, London, UK. 14. Hannover Medical School, Department of Conservative Dentistry, Periodontology and Preventive Dentistry, Hannover, Germany. 15. University of Szeged, Faculty of Dentistry, Szeged, Hungary. 16. Private practice, Valencia, Spain. 17. Birmingham Children's Hospital, UK.
Abstract
BACKGROUND: Inherited epidermolysis bullosa (EB) is a genetic disorder characterized by skin fragility and unique oral features. AIMS: 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.
BACKGROUND: Inherited epidermolysis bullosa (EB) is a genetic disorder characterized by skin fragility and unique oral features. AIMS: 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.
Authors: K Martin; S Geuens; J K Asche; R Bodan; F Browne; A Downe; N García García; G Jaega; B Kennedy; P J Mauritz; F Pérez; K Soon; V Zmazek; K M Mayre-Chilton Journal: Orphanet J Rare Dis Date: 2019-06-11 Impact factor: 4.123
Authors: D T Greenblatt; E Pillay; K Snelson; R Saad; M Torres Pradilla; S Widhiati; A Diem; C Knight; K Thompson; N Azzopardi; M Werkentoft; Z Moore; D Patton; K M Mayre-Chilton; D F Murrell; J E Mellerio Journal: Br J Dermatol Date: 2021-11-25 Impact factor: 11.113
Authors: Susanne Krämer; James Lucas; Francisca Gamboa; Miguel Peñarrocha Diago; David Peñarrocha Oltra; Marcelo Guzmán-Letelier; Sanchit Paul; Gustavo Molina; Lorena Sepúlveda; Ignacio Araya; Rubén Soto; Carolina Arriagada; Anne W Lucky; Jemima E Mellerio; Roger Cornwall; Fatimah Alsayer; Reinhard Schilke; Mark Adam Antal; Fernanda Castrillón; Camila Paredes; Maria Concepción Serrano; Victoria Clark Journal: Spec Care Dentist Date: 2020-11