Epidermolysis bullosa in oral health: clinical manifestations and salivary alterations

• Published in: Clinical oral investigations Vol. 27; no. 6; pp. 3117 - 3124
• Main Authors: de Azevedo, Brenda Lamônica Rodrigues, Roni, Gabriel Marim, Dettogni, Raquel Spinassé, Torrelio, Rosalie Matuk Fuentes, Leal, Lucas Fernandes, da Gama-de-Souza, Letícia Nogueira
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Berlin Heidelberg 01.06.2023; Springer Nature B.V
• Subjects: Cleft lip/palate; Dentistry; Disease; Epidermolysis Bullosa; Hereditary diseases; Hospitals; Humans; Medicine; Morphology; Mouth; Mouth Mucosa; Mucosa; Oral Health; Oral hygiene; Patients; Plastic surgery; Saliva; Science; Skin; Sociodemographics; Blisters; Epidermolysis bullosa; Saliva; Oral mucosa
• ISSN: 1436-3771; 1432-6981; 1436-3771
• DOI: 10.1007/s00784-023-04917-3

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.