Dental and Oral Health in hEDS
See Surgical Considerations for Anesthesia
Dental and oral health complications are common in individuals with hypermobile Ehlers-Danlos syndrome (hEDS) and can significantly impact quality of life. These issues are closely tied to the underlying connective tissue fragility caused by abnormal collagen, and they manifest in both the soft tissues (e.g., gums and mucosa) and hard structures (e.g., teeth, jaw joints, and bone). A multidisciplinary and preventive approach is essential for effective management.
Key Features
People with hEDS may experience
Fragile, bleeding gums
Crowded or misaligned teeth
High-arched, narrow palate
TMJ (temporomandibular joint) dysfunction
Poor healing after dental work
Frequent dental complications, including bruxism, dry mouth (xerostomia), and local anesthetic resistance
Causes and Mechanisms
Empirically Observed Causes
Abnormal collagen synthesis leading to weak periodontal and mucosal structures
Craniofacial growth abnormalities contributing to high palate and crowded teeth
TMJ joint hypermobility causing frequent subluxation or dislocation
Delayed or impaired healing following dental procedures
Increased mucosal and periodontal fragility, resulting in easy tearing or bruising
Xerostomia (dry mouth), possibly linked to autonomic dysfunction or medications
Poor response to local anesthetics during dental work
Increased debris index and larger distance between the cementoenamel junction and marginal bone, indicating higher periodontal disease risk
Theoretical Mechanisms
Altered proprioception affecting bite, chewing patterns, and jaw alignment
Myofascial dysfunction contributing to oral and jaw pain
Increased susceptibility to oral trauma due to tissue laxity
Pathological wound healing pathways specific to connective tissue disorders
Central sensitization possibly contributing to persistent or exaggerated pain responses
Microvascular fragility impacting gingival perfusion and tissue resilience
Observed Symptoms and Dental Findings
Soft Tissue Issues
Fragile gingiva prone to tearing, bleeding, and inflammation
Gingival recession and mucosal irritation even with gentle brushing
Larger-than-normal distance between the cementoenamel junction and marginal bone, increasing periodontal disease risk
Tooth and Jaw Development
High-arched, narrow palate, often with crossbite or speech issues
Dental crowding due to altered jaw structure
Greater risk of tooth root resorption and orthodontic relapse
TMJ Dysfunction
Pain in the jaw (myalgia [muscle pain] and arthralgia [joint pain])
Clicking, popping, or locking of the joint
Frequent disc displacements or recurrent dislocations
Restricted mouth opening and facial fatigue
Other Frequently Reported Symptoms
Xerostomia (dry mouth), possibly related to autonomic dysfunction or medication side effects
Poor efficacy of local anesthetics, requiring alternative strategies during dental work
Complications with extractions or implants, including prolonged healing or post-procedure pain
Treatment and Management
Preventive Dental Care
Meticulous oral hygiene is critical to prevent complications, with
Soft-bristled toothbrushes
Gentle flossing or use of water flossers
Fluoride rinses and non-alcoholic mouthwashes
Regular dental exams and cleanings—every 3–6 months if possible
Educate dental providers about hEDS and request gentle techniques
Managing Periodontal Disease
Follow standard periodontal protocols, but be alert to early-onset or severe disease
If periodontitis is aggressive and not explained by oral hygiene or lifestyle, evaluate for periodontal EDS, a distinct subtype with more severe dental involvement (see below)
TMJ Dysfunction Management
Conservative treatment is preferred, including
Physical therapy focused on jaw stabilization
Occlusal splints or nightguards for bruxism
Anti-inflammatory medications and muscle relaxants (short term)
Avoid excessive jaw opening during dental work
Surgery is high-risk due to tissue fragility and should be a last resort
Other Considerations
For dry mouth: sugar-free lozenges, saliva substitutes, and hydration
If local anesthetic fails, consider nerve blocks or alternative techniques
Topical anesthetics before cleanings
May help reduce pain from sensitive gums and fragile mucosa
Especially useful for patients with tissue fragility or oral hypersensitivity
Short-acting and may need reapplication during long procedures
Be mindful of potential allergic reactions, especially in patients with suspected mast cell activation
Not all patients respond to topicals; anesthetic resistance is possible
Monitor healing closely after any procedure—delayed recovery is common
Practical Tips
For Patients with hEDS
Inform your dentist that you have hypermobile Ehlers-Danlos syndrome
Use a soft-bristled toothbrush and non-abrasive toothpaste
Floss gently or use a water flosser to avoid gum trauma
Consider using fluoride rinses and alcohol-free mouthwash
Schedule dental cleanings more frequently (every 3–6 months)
Avoid opening your mouth too wide during dental care
Consider taking an anti-inflammatory before dental procedures if approved by your doctor
Report unusual or prolonged pain, poor healing, or numbness promptly
Use nightguards or occlusal splints if you grind or clench your teeth
For Dental Providers
Use gentle manipulation, retraction, and instrumentation to protect fragile tissues
Be cautious with prolonged procedures—schedule breaks and support the jaw
Understand that local anesthetic may be less effective—consider alternatives
Anticipate delayed healing and monitor recovery carefully
Avoid aggressive periodontal or surgical interventions when possible
Use a team-based approach: collaborate with physicians and physical therapists if needed
Document patient reports of TMJ instability, anesthetic resistance, or abnormal healing
Watch for signs of periodontal EDS if there is early-onset or severe periodontitis not explained by hygiene
Summary
Dental and oral health in hEDS is a significant but often underrecognized concern. Fragile tissues, abnormal craniofacial development, TMJ dysfunction, and healing complications can all contribute to frequent dental problems and reduced quality of life unless effectively managed. Preventive care, gentle dental techniques, and appropriate use of interventions—including topical anesthetics when helpful—can substantially improve comfort and outcomes.
Periodontal EDS
Periodontal Ehlers-Danlos Syndrome (pEDS) is a rare, inherited connective tissue disorder distinguished by severe and early-onset gum disease (periodontitis) and a generalized lack of attached gingiva—the gum tissue that usually anchors teeth. These two oral features are hallmarks of the condition, often appearing in childhood and progressing with age.
Unlike other forms of EDS, pEDS primarily affects the mouth, but it can also involve the skin, joints, blood vessels, and other systems. The condition is autosomal dominant, meaning it is passed down through families and caused by mutations in the C1R or C1S genes, which regulate part of the immune system (the classical complement pathway).
Key Features
Oral Signs
Lack of attached gingiva: A consistent and early sign, even before tooth loss
Severe periodontitis: Often starts around age 14, leading to rapid tooth loss in teens or early adulthood
Gingival recession: Common and nearly universal in adults
Other findings: Fragile gums, bleeding, and radiographic anomalies like fused roots or taurodontism
Systemic Signs
Easy bruising and pretibial plaques (dark patches on the shins)
Skin fragility and slow wound healing
Distal joint hypermobility (fingers/toes more than large joints)
Hoarse or high-pitched voice, sometimes marfanoid features
Rare complications: Vascular aneurysms, organ rupture, and white matter changes in the brain
Diagnosis
Diagnosis is based on clinical criteria, especially oral features, plus family history. In children, the absence of attached gingiva alone—especially with a positive family history—can support early suspicion. Genetic testing for mutations in C1R or C1S is used to confirm the diagnosis.
Management
Dental Care
Early and frequent periodontal care is critical
Tooth retention is prioritized due to poor outcomes with implants or dentures
Patients require cleanings every 3–6 months, soft toothbrushes, and tailored hygiene routines
Multisystem Support
May include rheumatology, dermatology, vascular care, physical therapy, and genetic counseling
Psychological support is also important due to the emotional impact of early tooth loss and systemic symptoms
Outlook
Without early intervention, most adults with pEDS lose their teeth prematurely, often by their 30s. Systemic complications are usually mild but can be serious. Quality of life is often affected by the physical, emotional, and social burden of the condition, making early diagnosis and multidisciplinary care essential.
