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Ehlers-Danlos syndrome: a case report.

N J Serman1, D A Albert

  • 1Division of Diagnostic Sciences, School of Dental and Oral Surgery, Columbia University.

Annals of Dentistry
|January 1, 1992
PubMed
Summary

Ehlers-Danlos syndrome Type VIII typically presents with periodontal issues, but this case shows delayed tooth eruption without them. This suggests a new subdivision (Type VIII B) for this rare dental presentation.

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Area of Science:

  • Dentistry
  • Genetics
  • Periodontology

Background:

  • Ehlers-Danlos syndrome (EDS) is a group of inherited connective tissue disorders.
  • Type VIII EDS is characterized by severe early-onset periodontitis.
  • Typical periodontal findings are often absent in some EDS variants.

Observation:

  • A 13-year-old male with Ehlers-Danlos syndrome Type VIII was evaluated.
  • The patient exhibited partially erupted deciduous teeth and mandibular permanent incisors.
  • Classic periodontal involvement associated with Type VIII EDS was notably absent.

Findings:

  • The case challenges the established presentation of Ehlers-Danlos syndrome Type VIII.
  • A potential subdivision, Type VIII B, is proposed for cases without periodontal involvement but with abnormal tooth eruption.
  • This subdivision contrasts with Type VIII A, which involves periodontal disease and normal eruption.

Implications:

  • This classification may refine understanding and diagnosis of Ehlers-Danlos syndrome.
  • It highlights the diverse oral manifestations of connective tissue disorders.
  • Further research is needed to validate the proposed subdivisions and their clinical significance.

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