Oral manifestations of Ehlers-Danlos syndromes

Am J Med Genet C Semin Med Genet. 2021 Dec;187(4):520-526. doi: 10.1002/ajmg.c.31941. Epub 2021 Nov 6.


Ehlers-Danlos syndromes (EDS) are a group of inherited connective tissue disorders. Patients with EDS exhibit distinct pathologies of the teeth and the oral cavity. Here, we summarize the current knowledge in the various EDS types, in particular regarding severe changes in oral health-related quality of life, the differential emergence of periodontitis, characteristic yet highly cumbersome dental manifestations, apparent anomalies of oral soft tissues, and relevant issues related to dental implantology. Resolution of remaining open questions will primarily rely on the standardization of diagnostic criteria. Clinical centers that specialize on this rare pathology need to apply congruent approaches for exact characterization of clinical features in conjunction with genetic validation that should be reached without exception in all patients and relevant family members.

Keywords: Ehlers-Danlos; hypermobility; periodontitis; pulp stones; root anomalies.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Connective Tissue Diseases*
  • Ehlers-Danlos Syndrome* / diagnosis
  • Ehlers-Danlos Syndrome* / genetics
  • Humans
  • Joint Instability*
  • Quality of Life
  • Skin Abnormalities*