Caries, Oral Hygiene, and Nutritional Habits in Hereditary Epidermolysis Bullosa

Objectives: Epidermolysis bullosa (EB) belongs to a group of hereditary disorders characterized by blistering of the skin. The classification in three major subgroups (simplex=EBS, junctional=EBJ, dystrophic=EBD) is based on the location of tissue cleavage and the different clinical features. The aim of this study was to determine the relationship between caries, poor oral hygiene, and nutritional habits in hereditary epidermolysis bullosa. Methods: 30 affected individuals of all three major groups (EBS: n=6, EBJ: n=3, EBD: n= 21) have been examined concerning oral soft and hard tissue lesions. Two questionnaires were created: one for the patient (nutrition habits) and one for the dentists (oral findings). Results: In EBS 15% of the patients have problems in swallowing food because of oral soft tissue involvement, throat and gullet. 50% of them eat only soft diet. 50% of the meals consist of sweets and cakes as a main dish. All patients consume juice or lemonade with sugar. 83,3% of the patients brush their teeth 1-2 times a day, 16,7% rarely. 83,3% have dental caries. All patients with EBJ prefer soft food (66,7% sweet diet) because of problems in swallowing. 66,7% of them drink lemonade or juice, all patients brush the teeth 1-2 times per day. All patients have dental caries. 80,9% of the patients with EBD consume only soft diet, 76,2% have problems in swallowing. 42,8% of the meals are sweet and 47,6% of the patients drink juice or lemonade. 42,7% brush their teeth 1-3 times a day, 42,8% rarely or never. Rampant dental caries is frequently seen in patients with EBD (100%). Conclusion: The excessive dental caries in EB is the result of a high sugar rate in a diet of soft and sticky consistency. The oral soft tissue involvement leads to a poor oral hygiene with a high risk of caries.