title: Hereditary epidermolysis bullosa: oral manifestations and dental management.
abstract: Epidermolysis bullosa (EB) is a diverse group of disorders that have as a common feature blister formation with tissue separation occurring at variable depths in the skin and/or mucosa depending on the specific EB type. There may be marked oral involvement, potentially creating devastating alterations in the soft and hard tissues. Oral tissue fragility and blistering is common to all EB types. However, oral debilitation as a result of soft tissue scarring is primarily limited to the recessive dystrophic EB subtypes. Generalized enamel hypoplasia appears to be limited to junctional EB, although rampant dental caries is associated with many individuals having generalized recessive dystrophic EB. While systemic treatment remains primarily palliative, it is possible to prevent destruction and subsequent loss of the dentition through appropriate interventions and dental therapy. The majority of individuals with mild EB subtypes may receive dental treatment with only minor modifications in approach. Even the most severely affected individuals with EB can retain their dentition using general anesthesia and conventional restorative techniques. With aggressive preventive interventions and management of developing malocclusions using serial extraction, it also is possible to reduce the likelihood of rampant caries, achieve an acceptable occlusion without the need for active tooth movement or appliance therapy, and allow these individuals to benefit from maintaining a natural healthy dentition.
authors.affiliation: Department of Pediatric Dentistry, The University of North Carolina at Chapel Hill.
authors.forename: J T
authors.initials: JT
authors.lastname: Wright
authors.forename: J D
authors.initials: JD
authors.lastname: Fine
authors.forename: L
authors.initials: L
authors.lastname: Johnson
mesh_terms: D000766:Anesthesia, Dental / Q000379:methods; D002648:Child; D002675:Child, Preschool; D017742:Dental Care for Chronically Ill; D003731:Dental Caries / Q000209:etiology; D003744:Dental Enamel Hypoplasia / Q000209:etiology; D004820:Epidermolysis Bullosa / Q000150:complications / Q000473:pathology; D006801:Humans; D009061:Mouth Mucosa / Q000473:pathology*