Dental treatment as a valuable adjunct in the interdisciplinary management of a child with severe dystrophic epidermolysis bullosa (Hallopeau-Siemens)

Severe dystrophic epidermolysis bullosa with oral involvement often leads to dental destruction and restricted food intake, resulting in malnutrition and maldevelopment. The patients become handicapped and have a poor prognosis. We report on a now 13-year-old Turkish child with normal secondary dentition who had severely damaged primary dentition. The teeth were treated surgically and then by continuous dental hygiene measures over the next 7 years. This care also resulted in an improvement in the nutritional state, associated anemia and the incidence of skin infections. Although cutaneous blistering and scarring has been progressive, with resulting mutilation of the fingers, the child is socially well adjusted in school and family. Its clinical course demonstrates that the fate of patients with severe dystrophic epidermolysis bullosa can be improved through multidisciplinary management.