Prevention of oral diseases in children with epidermolysis bullosa: a case report and literature review
DOI:
https://doi.org/10.15574/SP.2023.131.126Keywords:
children, epidermolysis bullosa, oral cavity, preventionAbstract
Today, more than 300 million people worldwide suffer from one or more rare diseases. Epidermolysis bullosa (EB) is a heterogeneous group of hereditary diseases characterized by a genetic predisposition to a hypersensitive body reaction to minor skin damage, characterized by the formation of blisters and erosions on the skin and mucous membranes, followed by scarring. It is one of the most severe hereditary dermatoses. In EB, pathological conditions also occur in the oral cavity. Thus, patients with EB require a special “atraumatic” approach to medical procedures, hygienic care, professional oral hygiene, etc.
Purpose - to systematize clinical recommendations for oral care for patients with BE on the basis of a clinical case.
Clinical case. We present a clinical case of a patient with a dystrophic EB and a diagnosis of acute chronic granulating periodontitis. Examination of the oral cavity revealed microstomia, multiple caries, erosions and ulcers on the oral mucosa, signs of chronic catarrhal gingivitis, as well as dentoalveolar anomalies. After achieving a satisfactory degree of mouth opening using a set of exercises for the maxillofacial muscles, treatment of the acute chronic granulating periodontitis of the tooth 23 was performed. As a result of the clinical observation and analysis of the literature, we systematized practical recommendations aimed at facilitating the treatment of oral diseases in patients with EB.
Conclusions. Treatment of oral diseases in patients with EB is primarily aimed at preventing pain and preventing secondary infection of affected areas. Treatment of dental caries contributes to the correct position of the tongue, improved swallowing and phonetics, as well as to improvement in nutritional status and aesthetic appearance. Oral hygiene reduces the risk of injury to the oral and pharyngeal mucosa. In the treatment of patients with EB, it is important to use a multidisciplinary approach with the involvement of specialists of different specialties. Implementation of simple but effective recommendations made by pediatric dentists will increase the effectiveness of prevention of oral diseases in these children.
The study was conducted in accordance with the principles of the Declaration of Helsinki. Informed consent of parents and child was obtained for the study.
No conflict of interests was declared by the authors.
References
Arpağ O, Arslanoğlu Z, Altan HO, Kale E, Bilgic F. (2015). Epidermolysis Bullosa in dentistry: Report of three cases and review of the literature. Journal of International Dental and Medical Research. 8: 133-139.
Babintseva AH, Hedeon IV, Hodovanets YuD, Hodovanets OS, Petrov VA. (2020). Buloznyi epidermoliz: klinichne sposterezhennia ta praktychni aspekty likuvannia. Aktualni problemy suchasnoi medytsyny: Visnyk Ukrainskoi medychnoi stomatolohichnoi akademii. 3: 9-13.
Brylinski M, Naderi M, Govindaraj RG, Lemoine J. (2018). Exploring the Opportunity Space to Combat Orphan Diseases with Existing Drugs. J Mol Biol. 15: 2266-2273. https://doi.org/10.1016/j.jmb.2017.12.001; PMid:29237557 PMCid:PMC5994353
Dag C, Bezgin TC, Ozalp N. (2014). Dental management of patients with epidermolysis bullosa. Oral health and dental management. 13: 623-627.
Datsenko IS, Kabachna AV, Hulpa VS. (2021). Suchasni pohliady na medychne zabezpechennia patsiientiv iz ridkisnymy khvorobamy systemy krovoobihu v ukraini i sviti. Farmatsevtychnyi zhurnal. 76; 4: 16-31.
Dawkins HJS, Draghia-Akli R, Lasko P, Lau LPL, Jonker AH, Cutillo CM et al. (2018). Progress in Rare Diseases Research 2010-2016: An IRDiRC Perspective. Clin Transl Sci. 11: 11-20. https://doi.org/10.1111/cts.12501; PMid:28796411 PMCid:PMC5759730
Derzhavnyi ekspertnyi tsentr MOZ Ukrainy. (2016). Adaptovana klinichna nastanova, zasnovana na dokazakh. Buloznyi epidermoliz: 77. URL: https://www.dec.gov.ua/wp-content/uploads/2019/11/2016_135_akn_be.pdf.
Dos Santos MN, Franco AG, dos Santos DS, da Silva WCG, Gomes LAB et al. (2022). Epidermolysis Bullosa: oral manifestations and dental management. Research, Society and Development. 11; 1: e40411125188. https://doi.org/10.33448/rsd-v11i1.25188
Fine JD, Bruckner-Tuderman L, Eady RAJ, Bauer EA, Bauer JW, Has C et al. (2014). Inherited epidermolysis bullosa: updated recommendations on diagnosis and classification. Dermatology. 70; 6: 1103-1126. https://doi.org/10.1016/j.jaad.2014.01.903; PMid:24690439
Fine JD, Hintner H (eds). (2009). Life with Epidermolysis bullosa. Etiology, Multidisciplinary Care and Therapy. Wien New York: Springer: 359. https://doi.org/10.1007/978-3-211-79271-1
Fine JD. (2010). Inherited epidermolysis bullosa. Orphanet J Rare Dis. 5: 12. https://doi.org/10.1186/1750-1172-5-12; PMid:20507631 PMCid:PMC2892432
Has C, Bauer JW, Bodemer C, Bolling MC, Bruckner-Tuderman L, Diem A et al. (2020). Consensus reclassification of inherited epidermolysis bullosa and other disoders with skin fragility. British Journal of Dermatology. 183; 4: 614-627. https://doi.org/10.1111/bjd.18921; PMid:32017015
Joseph C, Marty M, Dridi SM, Verhaeghe V, Bailleul-Forestier I, Chiaverini C et al. (2023). Oral health status in patients with inherited epidermolysis bullosa: a comparative multicenter study. Quintessence International. 54; 1: 34-43. doi: 10.3290/j.qi.b3479975
Kotvitska AA, Cherkashyna AV. (2016). Doslidzhennia suchasnoho stanu derzhavnoho rehuliuvannia medyko-farmatsevtychnoho zabezpechennia khvorykh na orfanni zakhvoriuvannia v Ukraini. Sotsialna farmatsiia v okhoroni zdorovia. 2; 1: 29-36. https://doi.org/10.24959/sphhcj.16.25
Krämer S, Lucas J, Gamboa F, Peñarrocha DM, Peñarrocha OD, Guzmán-Letelier M et al. (2020). Clinical practice guidelines: Oral health care for children and adults living with epidermolysis bullosa. Spec Care in Dentistry. 40: 3-81. https://doi.org/10.1111/scd.12511; PMid:33202040 PMCid:PMC7756753
Krämer S, Serrano MC, Zillmann G, Gálvez P, Araya I, Yanine N et al. (2012). Oral Health Care for Patients with Epidermolysis Bullosa - Best Clinical Practice Guidelines. International Journal of Paediatric Dentistry. 22: 1-35. https://doi.org/10.1111/j.1365-263X.2012.01247.x; PMid:22937908
Krämer S, Villanueva J (eds). (2011). Oral Health Care for Patients with Epidermolysis Bullosa: Best Clinical Practice Guidelines. DEBRA Int.: 66. https://doi.org/10.1111/j.1365-263X.2012.01247.x; PMid:22937908
Scheidt L, Sanabe ME, Diniz MB. (2015). Oral Manifestations and Dental Management of Epidermolysis Bullosa Simplex. Int J Clin Pediatr Dent. 8; 3: 239-241. https://doi.org/10.5005/jp-journals-10005-1321; PMid:26604545 PMCid:PMC4647048
Schieppati A, Henter JI, Daina E, Aperia A. (2008). Why rare diseases are on important medical and social issue. The Lancet. 9629: 2039-2041. https://doi.org/10.1016/S0140-6736(08)60872-7; PMid:18555915
Wiebe CB, Penagos H, Luong N, Slots J, Epstein E, Siegel D et al. (2003). Clinical and Microbiologic Study of Periodontitis Associated with Kindler Syndrome J Periodontol. 74: 25-31. https://doi.org/10.1902/jop.2003.74.1.25; PMid:12593592
Wiener RC, Dinsmore RR, Meckstroth R, Marshall W. (2016). Providing Daily Oral Infection Control to Persons Dependent on others for Activities of Daily Living: A Semi-Qualitative Descriptive Study. J Dent Craniofac Res. 1 (1): 1-11. https://doi.org/10.21767/2576-392X.100002; PMid:28191548 PMCid:PMC5303006
Wright JT. (1999). Oral manifestations of epidermolysis bullosa. In: Epidermolysis bullosa: clinical, epidemiologic, and laboratory advances, and the findings of the National Epidermolysis Bullosa Registry. Baltimore: Johns Hopkins University Press: 236-257.
Wright JT. (2010). Oral manifestations in the epidermolysis bullosa spectrum. Dermatol Clin. 28; 1: 159-164. https://doi.org/10.1016/j.det.2009.10.022; PMid:19945630 PMCid:PMC2787479
Downloads
Published
Issue
Section
License
Copyright (c) 2023 Modern pediatrics. Ukraine
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
The policy of the Journal “MODERN PEDIATRICS. UKRAINE” is compatible with the vast majority of funders' of open access and self-archiving policies. The journal provides immediate open access route being convinced that everyone – not only scientists - can benefit from research results, and publishes articles exclusively under open access distribution, with a Creative Commons Attribution-Noncommercial 4.0 international license (СС BY-NC).
Authors transfer the copyright to the Journal “MODERN PEDIATRICS. UKRAINE” when the manuscript is accepted for publication. Authors declare that this manuscript has not been published nor is under simultaneous consideration for publication elsewhere. After publication, the articles become freely available on-line to the public.
Readers have the right to use, distribute, and reproduce articles in any medium, provided the articles and the journal are properly cited.
The use of published materials for commercial purposes is strongly prohibited.