Dentine dysplasia with sclerotic bone and skeletal anomalies inherited as an autosomal dominant trait. A new syndrome

Oral Surg Oral Med Oral Pathol. 1977 Feb;43(2):267-83. doi: 10.1016/0030-4220(77)90163-3.


Patients with teeth showing all of the clinical, radiographic, and histologic features of radicular dentine dysplasia (type I) have been found to have dense sclerotic bone and skeletal anomalies of the wrists and hand bones. The association of these defects of teeth and bone was found to be transmitted as an autosomal dominant trait over four generations. Review of theliterature failed to disclose a previous description of this disorder.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Bone and Bones / abnormalities*
  • Bone and Bones / diagnostic imaging
  • Chromosomes
  • Dentin Dysplasia / diagnostic imaging
  • Dentin Dysplasia / genetics*
  • Dentin Dysplasia / pathology
  • Female
  • Genes, Dominant
  • Humans
  • Male
  • Osteosclerosis / diagnostic imaging
  • Osteosclerosis / genetics*
  • Radiography
  • Tooth Root / abnormalities