Background: Epidermolysis Bullosa (EB) is a rare disease with multiple oral manifestations requiring a special approach from the dentist. The scientific literature is scarce, making it difficult for dentists with no experience on EB to know how to approach patients in a safe manner. The present guideline has been developed using a standard methodology.

Objective: Provide the users with information on the special care people with EB require before, during and after dental treatment.

Methods: A systematic review of the literature was performed. The information gathered was discussed at a two day consensus meeting including dentists from different countries and patient representatives. To formulate the recommendations the SIGN system was used. The draft document was sent for external review to dental professionals and patient groups.

Results: Key recommendations include: A preventative protocol is the dental management approach of choice. Patients with EB should be referred to the dentist for the first consultation at the age of 3 to 6months. A dietary caries-prevention programme should be instigated at early age. Patients with the severe generalized RDEB subtype of EB require the most treatment modifications. Extreme care of fragile tissues is important: little pressure (compressive forces) can be applied, but no sliding movements should be used. Lips should be lubricated before any procedure is performed. Bullae occurring during treatment need to be drained to avoid spreading. Sutures can be used safely in all patients.

Conclusions: This guideline will help dentists treating patients with EB to provide safe and evidence based treatments.