Evaluation of Clinical and Oral Findings in Patients with Epidermolysis bullosa

Evaluation of Clinical and Oral Findings in Patients with Epidermolysis bullosa

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. In this prospectiv...

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Published in:Medicina (Kaunas, Lithuania) Vol. 59; no. 7; p. 1185
Main Authors: Yavuz, Yasemin, An, Isa, Yazmaci, Betul, Akkus, Zeki, Ortac, Hatice
Format: Journal Article
Language:English
Published: Switzerland MDPI AG 21-06-2023
MDPI
Subjects:
Anemia
bullae
Care and treatment
Collagen
Dental caries
Dental enamel
Dental health
Dysphagia
Epidermolysis bullosa
Esophagus
Families & family life
Gender
Genetic aspects
Health aspects
Kidney diseases
Malnutrition
Medical research
Medicine, Experimental
Mutation
oral hygiene
Skin
Toiletries industry
Wound healing
dental caries
bullae
oral hygiene
epidermolysis bullosa
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Summary: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. 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. 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. 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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ISSN:1648-9144
1010-660X
1648-9144
DOI:10.3390/medicina59071185