Epidermolysis bullosa in oral health: clinical manifestations and salivary alterations

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.