Dental management of patients with recessive dystrophic epidermolysis bullosa:

Abstract

Introduction: Epidermolysis bullosa (EB) is a group of rare inherited disorders uniquely characterised by skin, and in some instances, mucosal blistering. In the most severe form of the disease, recessive dystrophic EB (RDEB), the blister-inducing split occurs below the lamina densa. Extensive scarring of the oral mucosa results in limited mouth opening, making speech and mastication difficult. At the same time, oral mucosal blisters often lead to patients restricting their diets to soft, and generally cariogenic, foods, and battling with oral hygiene practices. This results in poor plaque control, a high caries burden and complex dental management. Aims and objectives: This paper presents a report on two siblings suffering from generalised RDEB affecting the oral cavity and their extensive dental treatment needs. Design/Methods: The siblings were referred to the University of Pretoria Oral Health Centre, complaining of painful teeth and oral mucosal discomfort. Their clinical features, dental condition and subsequent management are presented. Results: Restricted mouth opening, limited personal plaque control, and diets limited to soft, carbohydrate-rich foods because of oral mucosal discomfort, resulted in extensive dental decay that required multiple extractions. Conclusions: The dental and anaesthetic management of patients with RDEB is complex, and due to the friable tissues, requires the most atraumatic care possible. Ideally, patients with this condition need to have early dental intervention where preventive programmes can be implemented to reduce the need for later extensive and complicated dental interventions. Maintenance of the patient’s oral health is essential to ensure adequate nutrition, yet, reducing the consumption of soft cariogenic diets which increased their caries risk.