Oral and mandibular manifestations in the Ehlers-Danlos syndromes

Am J Med Genet C Semin Med Genet. 2017 Mar;175(1):220-225. doi: 10.1002/ajmg.c.31541. Epub 2017 Feb 13.


The Ehlers-Danlos syndromes (EDS) are hereditary disorders that affect the connective tissue and collagen structures in the body. Several types of EDS have been identified. Oral and mandibular structures, which include oral soft tissue, dentition, facial and head pain, and the functioning of the temporomandibular joint (TMJ), are variably affected in the various types of EDS. These various manifestations of EDS have been noted for many years, but newer diagnostic techniques and studies are shedding additional light on the challenges faced by EDS patients in the area of oral and mandibular disorders. Further, the impact of temporomandibular disorder (TMD) on musculoskeletal dysfunction and vice versa, make this an important feature to recognize. Oral and mandibular hypermobility of the TMJ with associated consequences of EDS are noted. These features, diagnostic parameters and treatment procedures are presented. © 2017 Wiley Periodicals, Inc.

Keywords: Ehlers-Danlos syndrome; hypermobility; oral manifestations; temporomandibular dysfunction; temporomandibular joint.

Publication types

  • Review

MeSH terms

  • Ehlers-Danlos Syndrome / pathology*
  • Ehlers-Danlos Syndrome / physiopathology
  • Humans
  • Joint Instability / physiopathology
  • Mandible / physiopathology
  • Mouth Diseases / pathology
  • Mouth Diseases / physiopathology
  • Temporomandibular Joint Disorders / diagnosis
  • Temporomandibular Joint Disorders / physiopathology