Recessive dystrophic Epidermolysis bullosa. Two case reports with 20-year follow-up

Abstract

These two case reports highlight the enormous clinical difficulties faced by dentists in providing satisfactory long-term dental care to patients who suffer from Epidermolysis bullosa. Problems of bullae formation in oral soft tissues and subsequent scarring are outlined in relation to the difficulty of maintaining satisfactory oral hygiene and a diet leading to minimal dental caries experience. The behavioural problems of maintaining patient compliance for preventive and restorative dentistry in this painful and debilitating disease are illustrated in these case reports. Difficulties in providing restorative care, either under local anaesthesia or general anaesthesia are discussed, and a novel replacement of non-viable carious anterior teeth using a nine-unit porcelain fused to metal Rochette type bridge is presented. Dental management of patients with Epidermolysis bullosa should commence at birth, and non-compliance in dental attendances should be followed up by social workers to prevent the disastrous oral morbidity that frequently occurs in such patients.