Epidermolysis bullosa (EB) is a group of hereditary or acquired disorders characterized by mechanical fragility of the skin and depending on the type and severity, may exhibit blistering of virtually any epithelial surface with subsequent scar formation and contractures. There are felt to be as many as 20 to 25 types of EB which have been classified into three major subgroups, based on the ultrastructural site of blistering: epidermolytic (EB simplex), junctional, a dermolytic (dystrophic EB and acquired EB). Enamel hypoplasia, rampant dental caries and oral blister formation with scarring have been associated with several types of EB. The long-term objective of this proposal is to elucidate the specific oral manifestations of the various types of EB and to develop appropriate recommendations for oral health care measures given the different disease patterns.
The specific aims of the study are to clinically determine the soft and hard oral tissue manifestations of the EB diseases; to determine if there is a relationship between the presence of enamel hypoplasia and dental caries; and to determine the ultrastructural changes in the enamel, dentin, and cementum of teeth in EB patients. The proposed study involves both clinical and laboratory investigations. Patients participating in the Southern Clinical Center for the National EB Registry at the University of Alabama at Birmingham will be evaluated and diagnosed using ultrastructural and immunofluorescence tissue mapping. Each individual will be clinically examined for soft tissue involvement of the oral and perioral structures. Areas of blister formation and/or scar tissue will be recorded. The dentition will be evaluated for variations in tooth morphology, the presence of enamel hypoplasia, and for quantitation of the dental caries experience. Photographs of the extra-oral and intra-oral tissues will be taken of each individual. Determination of the ultrastructural dental anomalies will be accomplished using light microscopy and scanning electron microscopy. Exfoliated primary teeth or teeth extracted for therapeutic reasons will be collected for histologic examination. The enamel, dentin, and cementum will be evaluated. Replicas of the labial enamel surface will be fabricated on each individual to establish which EB types demonstrate enamel defects. Study of the enamel replicas will also allow the enamel defects to be accurately characterized.
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