Abstract

Introduction: Junctional Epidermolysis Bullosa (JEB) is a reces- sive inherited disease that causes the mucous membrane and skin to be very fragile and to blister and bleed easily. In the mouth, JEB-affected children may present microstomia, peri-oral ulcera- tions, generalised hypoplasias and increased risk of caries. Early dental prevention is a key step if we want to keep appropriate oral health and reduce the suffering of children affected by JEB. Case report: A 10-month-year-old girl diagnosed with JEB in DEBRA foundation was referred to the Specialization Program in Paediatric Dentistry at University of Talca. First oral examination showed non-erupted teeth. A preventive stage was undertaken including healthy diet and oral hygiene. At 14-months follow-up, erupted teeth presented enamel defects with no-sign of dental car- ies. Therefore, fluoride toothpaste (1100 ppm), trimestral application of fluoride varnish (22.600 ppm) and glass ionomer sealants were indicated complementing previous preventive step. At second follow-up visit (6-months later), the behavioural problems, pres- ence of peri-oral ulceration and marked restriction of mouth open- ing made oral hygiene difficult. So, it was decided to apply silver diamine fluoride on erupted teeth to reinforce enamel structure and prevent caries formation. Currently, she is a caries-free 3-year-old patient with good behaviour during dental treatment that reduces bleeding from peri-oral lesions during dental procedures. Comments: This case validates the early dental prevention (before tooth eruption) by a paediatric dentist as a fundamental step to keep good oral health, reducing peri-oral ulceration/bleeding and favouring good quality of life in JEB-patients.