The Chemical Composition of Tooth Enamel in Recessive Dystrophic Epidermolysis Bullosa: Significance with Respect to Dental Caries

Abstract

Previous reports have linked the prevalence of tooth abnormalities with high caries experience in the different types of epidermolysis bullosa (EB). However, it is not known to what extent the apparent susceptibility to enamel caries is due to disease-related altered enamel chemistry in these cases. The aim of this study was to characterize the enamel of teeth from patients suffering from recessive epidermolysis bullosa dystrophica (rEBD) in terms of its mineral content, carbonate content, protein content, and amino acid composition. The results showed that dental enamel from these patients was essentially normal in terms of its chemistry. It is therefore concluded that the high caries experience in recessive dystrophic epidermolysis bullosa patients is probably related to other factors, such as compromised oral hygiene and prolonged oral clearance due to extensive oral soft tissue damage and a cariogenic diet.

Systemic disorders and their influence on the development of dental hard tissues: A literature review

“…Minor enamel defects have been shown to occur in all EB subtypes, with the most substantial occurring in the EBJ subgroup. [87][88][89][90] All patients with EDJ have been reported to present with defects in enamel, whereas the prevalence of defects in EBS and EBD was similar to that of the control population (27%), suggesting that the mechanism causing damage to enamel may be very different between the three subtypes. 88 In addition, Wright et al 88 reported that the incidence of dental caries was increased in patients with EBJ and EBD.…”

“…Studies into the chemical composition of enamel from EB patients, in terms of mineral content, carbonate content, protein content and amino acid composition, have reported essentially normal enamel chemistry in EBD patients whereas EBJ enamel contained a significantly reduced mineral per volume content, which resulted in enamel hypoplasia. 87,89 The high caries incidence in EBD patients may be related to other factors such as compromised oral hygiene, whereas in EBJ patients the enamel is developmentally compromised and associated with the genetic basis of the disease. In contrast, other workers have reported minor enamel defects in all three EB subtypes with a slight reduction (10%) in mineral content in some EBJ and EBD teeth.…”

Epidermolysis Bullosa Oropharyngeal Severity (EBOS) score: A multicenter development and reliability assessment

“…[7][8][9][10] Similarly, oral hard tissues may show either a marked developmentally compromised enamel or minor structural defects with areas of surface pitting and furrowing. [11][12][13][14][15] Although until now no consensus statement on EB severity score has been established, two reports have been published aimed at developing an EB scoring system. 16,17 These attempted to develop a method of scoring EB severity but evaluated too many variables (eg, skin, height, weight, mucous membranes, nutritional status, cancer) and gave little, if any, weight to the oropharyngeal component.…”

Type XVII Collagen is a Key Player in Tooth Enamel Formation

“…4 Non-Herlitz junctional epidermolysis bullosa (nH-JEB) caused by COL17 deficiency shows the abnormal tooth formation of amelogenesis imperfecta. [5][6][7] We therefore hypothesized that COL17 in hemidesmosomes also plays an important role in mesenchymal-epithelial interactions in tooth formation.…”