Periodontal manifestations of Ehlers-Danlos syndromes: A systematic review

J Clin Periodontol. 2017 Nov;44(11):1088-1100. doi: 10.1111/jcpe.12807. Epub 2017 Sep 25.


Aim: Ehlers-Danlos syndromes (EDS) are a group of inherited connective tissue disorders, characterized by joint hypermobility, skin hyperextensibility, and tissue fragility. Periodontal EDS (pEDS) is a specific EDS subtype caused by heterozygous mutations in complement 1 subunit genes C1R and C1S, with early severe periodontitis as predominant clinical feature. We aimed to systematically assess the spectrum of periodontal abnormalities in all EDS subtypes.

Materials and methods: An electronic and manual search was conducted in three databases (Medline, LIVIVO, CENTRAL). Publications of all study designs written in English/German without date restriction evaluating periodontal features in EDS were included.

Results: Thirty articles on pEDS and thirteen articles on other EDS subtypes were analysed. In pEDS, early severe periodontitis (98.4%) and gingival recession (87.1%) are the predominant features. Reports on periodontal manifestations in other EDS subtypes are rare. Described were severe gingival enlargement in dermatosparaxis EDS, and localized periodontal breakdown related to teeth with shortened roots in classical EDS (n = 3, respectively).

Conclusion: Early severe periodontitis is the hallmark of pEDS; there is no evidence that it is part of the clinical phenotype of other EDS subtypes. Stringent analyses of periodontal manifestations in most EDS subtypes are missing. Prospero registration number CRD42017056889.

Keywords: Ehlers-Danlos; gingiva; hypermobility; oral manifestations; periodontitis.

Publication types

  • Review
  • Systematic Review

MeSH terms

  • Ehlers-Danlos Syndrome / complications
  • Ehlers-Danlos Syndrome / pathology*
  • Gingiva / pathology
  • Gingival Recession / etiology
  • Humans
  • Periodontitis / etiology
  • Periodontium / pathology*