The formation and homeostasis of the mineralized tissues that make up the tooth and its periodontium show many similarities, but also differences, with respect to bone tissue. Accordingly, oral manifestations are often present in skeletal diseases. The manifestations in X-linked hypophosphatemia (XLH) are a good illustration of the potential impact of skeletal diseases on the teeth and periodontium: they can affect all oral mineralized tissues; they differ between children and adults; and they have a strong impact on the quality of life of the affected individual. In addition, the frequency and severity of the oral manifestations depend on the general management of the skeletal disease. The main dental tissues affected by XLH are the dentin and cementum, whose formation and mineralization are impaired. Clinically, poorly mineralized dentin leads to spontaneous endodontic infections, and dental abscesses in seemingly intact teeth are frequent in affected children. Reduced cementum formation results in periodontal attachment loss, and the prevalence and severity of periodontitis are elevated in adults with XLH. Prevention or improvement of these dental manifestations is first achieved by conventional treatment of XLH with active vitamin D analogs and phosphate supplementation. In addition, local treatments preventing bacterial invasion of dentin are beneficial.
