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Evaluation of Clinical and Oral Findings in Patients with Epidermolysis bullosa

Introduction: Epidermolysis bullosa (EB) is a genetically inherited disease characterized by recurrent bullae and erosions on the skin with numerous signs of dental caries and poor oral hygiene. The aim of this study was to investigate the general clinical and oral findings of patients with EB. Mate...

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Autores principales: Yavuz, Yasemin, An, Isa, Yazmaci, Betul, Akkus, Zeki, Ortac, Hatice
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10385745/
https://www.ncbi.nlm.nih.gov/pubmed/37511997
http://dx.doi.org/10.3390/medicina59071185
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author Yavuz, Yasemin
An, Isa
Yazmaci, Betul
Akkus, Zeki
Ortac, Hatice
author_facet Yavuz, Yasemin
An, Isa
Yazmaci, Betul
Akkus, Zeki
Ortac, Hatice
author_sort Yavuz, Yasemin
collection PubMed
description Introduction: Epidermolysis bullosa (EB) is a genetically inherited disease characterized by recurrent bullae and erosions on the skin with numerous signs of dental caries and poor oral hygiene. The aim of this study was to investigate the general clinical and oral findings of patients with EB. Materials and Methods: In this prospective study, the clinical and oral findings and family history of 26 cases with EB were evaluated. The type of EB, gender, age, parental consanguinity, dental caries, oral findings, distribution of lesions and presence of associated anomalies, clinical and oral findings correlated with gender were recorded. Results: All 26 patients with EB had a history of consanguinity and siblings with EB to varying degrees. In our study, malnutrition, anemia and growth retardation, gastrointestinal system complications, hair thinning, hand and nail deformity, ocular problems and renal disease (in one case) were observed with variable frequencies. When the intraoral findings of the patients were investigated, extensive dental caries in all EB types, enamel hypoplasia in junctional EB (JEB) and the presence of tooth-root to be extracted in dystrophic EB (DEB), intraoral bullae and lesions, ankyloglossia, vestibular sulcus insufficiency, microstomia and maxillary atrophy were observed. Three cases had restorative treatment and one case had prosthetic rehabilitation. Conclusions: Oral involvement can be seen with varying frequencies depending on the type of EB and the severity of the disease. It may result from delayed oral and dental rehabilitation due to physical disabilities, limitations and more pressing medical problems. Microstomy, pain from mucosal lesions, and restricted access to the mouth can be caused by poor oral hygiene. Oral complications and caloric needs of individuals with EB should be determined, and individual prophylaxis should be applied to prevent caries formation and protect teeth.
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spelling pubmed-103857452023-07-30 Evaluation of Clinical and Oral Findings in Patients with Epidermolysis bullosa Yavuz, Yasemin An, Isa Yazmaci, Betul Akkus, Zeki Ortac, Hatice Medicina (Kaunas) Article Introduction: Epidermolysis bullosa (EB) is a genetically inherited disease characterized by recurrent bullae and erosions on the skin with numerous signs of dental caries and poor oral hygiene. The aim of this study was to investigate the general clinical and oral findings of patients with EB. Materials and Methods: In this prospective study, the clinical and oral findings and family history of 26 cases with EB were evaluated. The type of EB, gender, age, parental consanguinity, dental caries, oral findings, distribution of lesions and presence of associated anomalies, clinical and oral findings correlated with gender were recorded. Results: All 26 patients with EB had a history of consanguinity and siblings with EB to varying degrees. In our study, malnutrition, anemia and growth retardation, gastrointestinal system complications, hair thinning, hand and nail deformity, ocular problems and renal disease (in one case) were observed with variable frequencies. When the intraoral findings of the patients were investigated, extensive dental caries in all EB types, enamel hypoplasia in junctional EB (JEB) and the presence of tooth-root to be extracted in dystrophic EB (DEB), intraoral bullae and lesions, ankyloglossia, vestibular sulcus insufficiency, microstomia and maxillary atrophy were observed. Three cases had restorative treatment and one case had prosthetic rehabilitation. Conclusions: Oral involvement can be seen with varying frequencies depending on the type of EB and the severity of the disease. It may result from delayed oral and dental rehabilitation due to physical disabilities, limitations and more pressing medical problems. Microstomy, pain from mucosal lesions, and restricted access to the mouth can be caused by poor oral hygiene. Oral complications and caloric needs of individuals with EB should be determined, and individual prophylaxis should be applied to prevent caries formation and protect teeth. MDPI 2023-06-21 /pmc/articles/PMC10385745/ /pubmed/37511997 http://dx.doi.org/10.3390/medicina59071185 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Yavuz, Yasemin
An, Isa
Yazmaci, Betul
Akkus, Zeki
Ortac, Hatice
Evaluation of Clinical and Oral Findings in Patients with Epidermolysis bullosa
title Evaluation of Clinical and Oral Findings in Patients with Epidermolysis bullosa
title_full Evaluation of Clinical and Oral Findings in Patients with Epidermolysis bullosa
title_fullStr Evaluation of Clinical and Oral Findings in Patients with Epidermolysis bullosa
title_full_unstemmed Evaluation of Clinical and Oral Findings in Patients with Epidermolysis bullosa
title_short Evaluation of Clinical and Oral Findings in Patients with Epidermolysis bullosa
title_sort evaluation of clinical and oral findings in patients with epidermolysis bullosa
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10385745/
https://www.ncbi.nlm.nih.gov/pubmed/37511997
http://dx.doi.org/10.3390/medicina59071185
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