The chemical composition of tooth enamel in junctional epidermolysis bullosa

The junctionalis form of epidermolysis bullosa (EBJ) is associated with a n umber of clinical problems involving tooth enamel, including increased susc eptibility to caries. The aim here was to carry out a chemical characteriza tion of the enamel of teeth from EBJ patients compared with that of unaffec ted controls. The results showed that while protein concentration, amino ac id composition and carbonate content were similar in both groups, EBJ ename l contained a significantly reduced mineral per volume content, resulting i n enamel hypoplasia. In addition. Western blotting revealed the presence of serum albumin (a known inhibitor of enamel crystal growth) in EBJ enamel. This was not detected in control enamel or in enamel of teeth from patients with the dystrophic form of the disease. It is concluded that EBJ enamel i s developmentally compromised and that the enamel defects are commensurate with the reported genetic lesions. (C) 2000 Elsevier Science Ltd. All right s reserved.