If this happens, the lesions have to be drained. Post-operative instruction must highlight that the patients should not bite, rub, or traumatize their lip while under the effect of local anaesthesia. The main benefits of local anaesthesia are that it maintains airway patency and

provides prolonged post-operative pain relief. Examples of successful treatments provided under local anaesthesia include multiple extractions, implants, root canal treatment, and restorations6,16,23. Some authors suggest that less mucosal damage is produced when patients are treated under local anaesthesia when compared to general anaesthesia. When planning a procedure under general anaesthesia, the patient’s this website MD/GP should be consulted13. The availability of an anaesthetic team with experience in EB is crucial. If this is not available, the use of local anaesthesia should be considered. Treatment under general anaesthesia allows the provision of extensive reconstructive dental treatment and multiple extractions regardless of the severity of soft tissue fragility and microstomia present5,7. The fact that the patient will be asleep, however, does not mean that the procedure will be easy to perform. Patients with severe fragility will still develop intra-operative generalized mucosal

sloughing secondary to retraction and minor trauma of the procedure itself1,7,36. Oral surgery and restorative procedures can be combined with other surgical procedures, as for example, oesophageal dilatation1. As stated previously, a water-soluble lubricant should be used instead of petrolatum in the operating Gamma-secretase inhibitor room because it is not flammable. A preventive protocol is today’s dental management approach of choice1,2. Patients with EB should be referred to the dentist for the first consultation at the age of 3–6 months. Tooth brushing is possible in all patients with EB, even in patients with

the severe generalized RDEB subtype. The following suggestions can help determine the appropriate toothbrush for each patient: (a)  Small head5,7,8,11,13. Gentle and careful ultrasonic scaler and polish techniques can be used in all patients, including severe RDEB11. Topical applications of high-dose fluoride varnish are suggested every 3 months in patients with high caries risk C-X-C chemokine receptor type 7 (CXCR-7) or at each dental visit5,7,19. For children resident in nonfluoridated communities, the importance of daily fluoride supplements has been highlighted10. A dietary caries-prevention programme should be instigated at early age16,18. It is essential that dentists and nutritionists collaborate on an appropriate programme for each patient, as opposed to giving contradictory advice that may confuse patients and parents/guardians. Patients with severe generalized RDEB should perform daily exercises to improve/maintain a good mouth opening. This can be performed, for example, during dressing changes.