Abstract

Introduction: Dystrophic Epidermolysis Bullosa (DEB) is a genetic rare disease characterized by skin and mucous membrane blistering at minor traction or trauma with multiple oral pheno- types. DEB requires gentle therapeutic approach because scar formation may generates tissue retraction, vestibular obliteration, ankyloglossia and microstom ıa, making oral rehabilitation much more complex. If prevention is not incorporated early in patient life, proper oral health can be a challenging aim to reach even after an apparent suitable rehabilitation approach. Case report: A 14-year-old boy with recessive DEB and signs of high anxiety and social risk was referred to Specialization Pro- gram in Pediatric Dentistry, Universidad de Talca, Chile. Clinical examination revealed an extremely fragile orofacial region and limited mouth opening, poor oral hygiene, extensive dental destruction and multiple residual tooth roots. After obtaining informed consent, preventive step included oral hygiene instruc- tion, dietary advice, sealants and fluoride varnish applications. Under nitrous oxygen inhalation (NOI) to manage dental anxi- ety, restorative step included, endodontic treatment, multiple den- tal extractions and aesthetic rehabilitation. Despite planning 3- months follow up, the patient has not attended to regular check up during the last 5 years. Family support, pain of intraoral region and severe pseudosindactilia complicated toothbrush and oral hygiene control. In last visits new active caries were observed and extractions of some molars have been indicated under NOI. Comments: Early incorporation of preventive habits in oral health care beside regular check up and family support are needed if we want the success of complex restorative approaches, especially in DEB-affected children.