Ehlers-Danlos Syndromes: History
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Subjects: Pathology
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Ehlers–Danlos syndromes (EDS) are a clinically and genetically heterogeneous group of hereditary connective tissue disorders involving joint hyperlaxity, cutaneous hyperelasticity, and tissue fragility.

  • Ehlers Danlos
  • Rare Diseases
  • oral health

1. Introduction

A revised classification containing 13 subtypes was published by the International EDS Consortium, with a number of clinical conditions to guide and improve the diagnosis of each subtype [2]. In the European Union, a disease is considered ‘rare’ if it affects fewer than one in two thousand people [3]. In the case of EDS, the prevalence varies, depending on the type: between 1:30,000 (classical type) and < 1:1,000,000 (arthrochalasia type), whereas for the rarest form (the periodontitis type), the prevalence is unknown [1].

EDS’ oral conditions have been described as periodontitis [2,4,5], temporomandibular dysfunctions [2,6], bleeding tendencies [7,8], enamel hypoplasia [2,9], shape abnormalities of the teeth and changes in the number of teeth [2,8], a high palate [2,10], dysgnathia, and malocclusion [2,11], as well as decreased effects of local anesthesia [12,13].

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This entry is adapted from the peer-reviewed paper 10.3390/medicina56090448

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