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Periodontal manifestations of Ehlers-Danlos syndromes : a systematic review

Kapferer-Seebacher, Ines (author)
Lundberg, Pernilla (author)
Umeå universitet,Institutionen för odontologi
Malfait, Franziska (author)
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Zschocke, Johannes (author)
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 (creator_code:org_t)
2017-09-25
2017
English.
In: Journal of Clinical Periodontology. - : John Wiley & Sons. - 0303-6979 .- 1600-051X. ; 44:11, s. 1088-1100
  • Research review (peer-reviewed)
Abstract Subject headings
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  • 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.

Subject headings

MEDICIN OCH HÄLSOVETENSKAP  -- Klinisk medicin -- Odontologi (hsv//swe)
MEDICAL AND HEALTH SCIENCES  -- Clinical Medicine -- Dentistry (hsv//eng)

Keyword

Ehlers-Danlos
gingiva
hypermobility
oral manifestations
periodontitis

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Kapferer-Seebach ...
Lundberg, Pernil ...
Malfait, Franzis ...
Zschocke, Johann ...
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MEDICAL AND HEALTH SCIENCES
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Umeå University

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