Prior to becoming chronically ill, I was a full-time dental hygienist for 12 years.  I was very passionate about my career.  My health and tremors cut my ability to do clinical hygiene short, but I am thankful for the knowledge and experience I gained through that time period. I have been out of practice since 2015 so maybe some things have changed, but I know when I was in school and participated in continuing education courses, Ehlers-Danlos Syndrome was never talked about.  For that matter, we rarely touched on any oral manifestations of any of the less common diseases.  It seems when disease-related oral manifestations were talked about it was always related to diabetes, and to periodontal disease in relation to heart disease.  I guess it makes sense to cover the more common problems we would be facing, and after all, there is only so much time to fit the curriculum in. In fact, in my 12 years of being a dental hygienist, I had never even heard of Ehlers-Danlos Syndrome (EDS), or of the other similar connective tissue diseases That I now have learned about.

During my chronic illness journey, I found out that chronic Lyme disease was not the one and only medical issue I was dealing with.  I had met with a geneticist initially for my son. He was having some chronic joint pain and his chiropractor noticed he had hypermobility in several joints and referred us to the geneticist for an EDS evaluation.  EDS is a lot of times an inherited condition.  The geneticist went through our family history and medical history.  Then they also checked my hypermobility as well.  Come to find out we both have a lot of symptoms of hypermobility EDS.  Later on, I took a genetics test and found out additionally I have a mutation in a gene that is related to collagen VI muscular dystrophies which actually has most of the same symptoms of hypermobility EDS.  A lot of times these diseases can overlap or present themselves in the same way.

Ehlers-Danlos Syndrome is a connective tissue disease typically characterized by tissue fragility, skin hyperelasticity, joint hyperlaxity, keloid scarring, velvety skin and can have vascular manifestations. Collagen defects cause problems for mouth health including teeth, gum tissue, nerve tissue, tendons, ligaments, and blood vessels. Each phenotype has some of its own special characteristics. Mouth and jaw symptoms have been found in every phenotype.

As of right now, there are 8 main known phenotypes of EDS. Type VIII happens to distinctly be related to oral manifestations. It is called Periodontal Ehlers-Danlos Syndrome.  This is the rarest form of EDS and is characterized by an early-onset periodontal disease and hyperpigmented atrophic scars in the pretibial zones.  The periodontitis will typically be generalized, cause alveolar bone loss, and possible loss of teeth prior to 30. Other oral characteristics may include: gingival hyperplasia, hyperkeratosis, micrognathia, agenesis or microdontia of multiple teeth, and sometimes increased infection risk.

These Collagen diseases often go undiagnosed and dental professionals are in a key position to pick up on some signs that a collagen disease may be to blame.

Extraoral Manifestations

Some extraoral manifestations that may be found could include: The presence of scarring on the face, hyperelasticity of skin, bruising, hypertelorism, epicanthus, and repeated subluxations of the TMJ.  A vaulted palate is common in EDS patients.  TMJ locking, pain, and laxity are common. People with EDS can have issues with getting numb from local anesthetic. This could possibly be due to the altered collagen affecting oxygen and nutrient diffusion through tissue.

Intraoral Manifestations

Intraoral manifestations may include: A fragile mucosa that tears easily, sutures may not hold well.  The fragility of the mucosa may cause hemorrhage issues during dental procedures.  Early-onset of periodontal disease along with recession may be found. Premolar and molar cusps may have extra deep fissures and long cusps.  Hypoplasia of enamel is common.  Microdontia is sometimes present and teeth seem to be fragile.  There may also be pulp stones or short deformed roots on radiographic examination. Up to 50% of EDS patients are able to touch the tip of their tongue to their nose this is called the

.

Gorlin sign. Lack of frenula has been noted in some cases.

Clinical Considerations

  • Some EDS patients have Mitral Valve Prolapse or other heart conditions, check if premedication is needed prior to dental treatment.  Patients with EDS may need a bite block used for their jaw to prevent TMJ issues and frequent breaks.  Neck and lumbar support in the chair may be needed as well.
  • Surgical procedures should be avoided if possible due to poor wound healing, sutures not holding well, and possible bleeding issues.  There is also an increased risk of endocarditis in patients with cardiac valve abnormalities.
  • Orthodontic treatment should be lighter because of the periodontal ligament being fragile.  Bracket placement needs to be away from tissue to avoid mucosal ulcerations. There is a higher chance of rapid movement, consider keeping retainers in longer.
  • There are no special considerations for patients with any type of prosthodontics or restorations such as crowns and fillings.
  • Endodontic treatment could be complicated if the person has pulp stones or root deformities.
  • There is not much evidence on dental implants on EDS patients, but few side effects are anticipated follow regular procedures.
  • Periodontal disease should be treated by a periodontal specialist.
  • Treatment and management of TMD associated problems depend on the cause.  Prevention of TMJ injury is important. Lifestyle changes can include: changing of chewing patterns, using mouth guards, muscle relaxers, anti-inflammatories, and pain medications.  Sometimes Botox injections can be used to relax muscles at trigger points.  Physical therapy may also be helpful.

In Closing

Research has confirmed there are oral manifestations associated with all forms of EDS.  Collagen VI muscular dystrophies are very closely related to EDS.  There is not a lot of evidence or studies out there talking about the oral manifestations of Collagen VI disorders, but it would stand to reason there may be some similarities to that of EDS patients considering they both affect collagen matrix. Certain clinical considerations need to be taken into account when dealing with a person who has EDS. If you are a patient with Ehlers-Danlos Syndrome, it is paramount to have great oral hygiene to avoid any unnecessary invasive treatment. If you are a dental caregiver and witness any of these classical signs of EDS, a consult with genetics, cardiology, and/or rheumatology may be indicated to confirm the diagnosis of this condition.

References

  1. Yves Letourneau DDS, Renald Perusse DDS, Helene Buithieu DDS. Oral Manifestations of Ehlers-Danlos Syndrome
  2. John Mitakides, Brad T. Tinkle, adapted by Benjamin Guscott. Oral and Mandibular Manifestations in the Ehlers-Danlos Syndromes
  3. Stephen Porter, Oral and Dental Implications of the Ehler-Danlos Syndromes
  4.  Orphanet, Orpha Number: 75392: Periodontal Ehlers-Danlos Syndrome