Trichodental dysplasia: a rare syndrome with distinct dental findings

Pediatr Dent. 2006 Jul-Aug;28(4):345-9.


The association of fine, dry, short hair and the developmental absence of several teeth has been associated with a rare autosomal dominant variant of ectodermal dysplasia known as trichodental syndrome or trichodental dysplasia. The purpose of this study was to present the case of a 4-year-old boy with trichodental syndrome. Clinical, radiographic, and genetic manifestations are described, along with a pertinent review of the literature.

Publication types

  • Case Reports

MeSH terms

  • Cariostatic Agents / therapeutic use
  • Child, Preschool
  • Crowns
  • Ectodermal Dysplasia / pathology
  • Fluorides / therapeutic use
  • Hair / abnormalities*
  • Humans
  • Karyotyping
  • Male
  • Microcephaly
  • Odontodysplasia / pathology*
  • Pedigree
  • Syndrome
  • Tooth Extraction


  • Cariostatic Agents
  • Fluorides