Epidermolysis bullosa in oral health: clinical manifestations and salivary alterations.

Autor: de Azevedo BLR; Graduate Program in Dental Science, Health Science Center, Federal University of Espírito Santo, Vitória, ES, Brazil., Roni GM; Medical School, Morphology Department, Health Science Center, Federal University of Espírito Santo, Vitória, Brazil., Dettogni RS; Morphology Department, Health Science Center, Federal University of Espírito Santo, Vitória, ES, Brazil., Torrelio RMF; Plastic Surgery, Hospital Infantil Nossa Senhora da Glória, Debra, Vitória, ES, Brazil., Leal LF; Faculdades Integradas Espírito Santenses (FAESA), Vitória, ES, Brazil., da Gama-de-Souza LN; Graduate Program in Dental Science, Morphology Department, Health Science Center, Federal University of Espírito Santo, Avenue Marechal Campos, 1468, Maruípe, 29.040-090, Vitória, ES, Brazil. leticia.souza@ufes.br.
Jazyk: angličtina
Zdroj: Clinical oral investigations [Clin Oral Investig] 2023 Jun; Vol. 27 (6), pp. 3117-3124. Date of Electronic Publication: 2023 Feb 13.
DOI: 10.1007/s00784-023-04917-3
Abstrakt: Epidermolysis bullosa (EB) is an inherited disease characterized by the fragility of the skin and mucous membranes. All types/subtypes of EB can lead to alterations in the mouth and glands.
Objective: To evaluate clinical manifestations of EB on the oral mucosa and alterations in salivary flow.
Materials and Methods: Sociodemographic and clinical data were obtained from EB individuals. The salivary flow analysis was performed in EB and in non-EB patients. Fischer's exact test was applied to the qualitative variables, and the Mann-Whitney test was applied to the quantitative data.
Results: A total of 11 cases of EB were evaluated, and 3 types of EB were diagnosed (recessive dystrophic-RDEB; junctional-JEB; and simplex-EBS). Only individuals with RDEB or JEB showed the oral manifestation of the disease. The most affected sites were the lips (54%), hard palate (36%), and oral mucosa (27%). Ulcer and ankyloglossia were diagnosed in all RDEB cases. Regarding salivary flow, an intragroup comparison revealed an increase in stimulated versus unstimulated collection in the control sample (p = 0.0064). The EB group showed no difference (p = 0.6086). We also observed no differences in salivary volume between the control and EB groups (p = 0.7117 and p = 0.5557, unstimulated and stimulated flows, respectively).
Conclusions: No oral manifestations were observed in EBS subjects. It is unclear whether individuals with EB are predisposed to manifest hyposalivation.
Clinical Relevance: Severe cases of EB show broad alterations in the oral mucosa, whereas the saliva needs to be better evaluated.
(© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)
Databáze: MEDLINE