Ehlers-Danlos Syndrome

Ehlers Danlos syndrome (EDS) is a group of hereditary connective tissue disorders that manifests clinically with skin hyperelasticity, hypermobility of joints, atrophic scarring, and fragility of blood vessels. In 2017, a new international classification of EDS proposed 13 different variants and classified into six types (classical, vascular, hypermobile, arthrochalasis, kyphoscoliotic, and dermatosparaxis), with each type caused by different collagen pathology.

 

Asymptomatic, nonsyndromic joint hypermobility, Ehlers-Danlos syndrome, and hypermobility spectrum disorders (particularly the hypermobile type) are the commonest phenotypes associated with joint hypermobility. Manifestations of these syndromes include chronic pain, dysautonomia, gastrointestinal dysmotility, mast cell activation, anxiety and phobic states.

 

Clinical recognition of EDS variants is important to guide management because of the multiple, different manifestations of the variants. For example, the vascular type  is associated with arterial rupture and organ perforation with potentially life-threatening consequences whereas other types display different manifestations and risks.

Although EDS is mainly diagnosed clinically, identifying the gene encoding the collagen or proteins interacting with it is necessary to identify the type of EDS.

 

Key to Links:

  • Grey text – handout
  • Red text – another page on this website
  • Blue text – Journal publication or outside website

Definitions and Terms Related to Pain

 

 

Resources

 

References

Ehlers-Danlos Syndrome (EDS) – Overviews

  1. Ehlers-Danlos-Syndrome – NIH
  2. Ehlers Danlos Syndrome – StatPearls – NCBI Bookshelf – 2023
  3. Hypermobility Disorders – An Update for Clinicians MYOPAIN 2013

 

Ehlers-Danlos Syndrome – Diagnosis

  1. EhlersDanlos Syndrome Testing
  2. Accuracy of Clinical Diagnostic Criteria for Patients With Vascular Ehlers-Danlos Syndrome in a Tertiary Referral Centre – 2019
  3. Diagnosis and management of vascular Ehlers-Danlos syndrome – 2023
  4. A multi-institutional experience in vascular Ehlers-Danlos syndrome diagnosis – 2019
  5. Mast cell disorders in Ehlers-Danlos syndrome – PubMed 2017

 

Ehlers-Danlos Syndrome – Vascular (vEDS)

  1. Diagnosis and management of vascular Ehlers-Danlos syndrome – 2023
  2. A multi-institutional experience in vascular Ehlers-Danlos syndrome diagnosis – 2019
  3. Accuracy of Clinical Diagnostic Criteria for Patients With Vascular Ehlers-Danlos Syndrome in a Tertiary Referral Centre – 2019

Ehlers-Danlos Syndrome – Gastrointestinal Manifestations

  1. Hypermobile Ehlers–Danlos syndrome and disorders of the gastrointestinal tract- What the gastroenterologist needs to know – 2021

 

Ehlers-Danlos Syndrome – Ketamine

  1. Reduction of central neuropathic pain with ketamine infusion in a patient with Ehlers–Danlos syndrome – a case report – 2016
  2. Biomarkers for Ehlers-Danlos Syndromes- There Is a Role – 2021

 

Ehlers-Danlos Syndrome – Magnesium

  1. Magnesium-and-Ehlers-Danlos-Syndrome Part 1 – 2013
  2. Magnesium-and-Ehlers-Danlos-Syndrome Part 2 – 2013

Ehlers-Danlos Syndrome – Nutrition

  1. Nutritional, Therapeutic Strategy for Ehlers–Danlos Syndrome
  2. A novel therapeutic strategy for Ehlers–Danlos syndrome based on nutritional supplements – 2005

 

Ehlers-Danlos Syndrome – Pain

  1. Chronic pain in patients with the hypermobility type of Ehlers–Danlos syndrome – evidence for generalized hyperalgesia – 2014
  2. Chronic pain in hypermobility syndrome and Ehlers–Danlos syndrome (hypermobility type) – it is a challenge – 2015
  3. Reduction of central neuropathic pain with ketamine infusion in a patient with Ehlers–Danlos syndrome – a case report – 2016
  4. Oxidation Sensitive Nociception Involved in Endometriosis Associated Pain – 2015
  5. Small fiber neuropathy is a common feature of Ehlers-Danlos syndromes – 2016
  6. Management of chronic pain in Ehlers–Danlos syndrome – 2018
  7. Pain in Ehlers–Danlos Syndrome- A Non-Diagnostic Disabling Symptom – 2023
  8. A review of the high-concentration capsaicin patch and experience in its use in the management of neuropathic pain 2013
  9. Use of complementary and alternative medicine by patients with hypermobile Ehlers–Danlos Syndrome- A qualitative study – 2022
  10. Multidisciplinary Approach to Treating Chronic Pain in Patients with Ehlers–Danlos Syndrome- Critically Appraised Topic – 2022
  11. Pain management in the Ehlers Danlos syndromes – 2017
  12. Ehlers-Danlos Syndrome- An Analysis of the Current Treatment Options – 2020

 

Ehlers-Danlos Syndrome – Small Fiber Neuropathy

  1. Small fiber neuropathy is a common feature of Ehlers-Danlos syndromes. – PubMed – NCBI
  2. Small fiber neuropathy is a common feature of Ehlers-Danlos syndromes – 2016

 

Ehlers-Danlos Syndrome – Treatment

  1. A review of the high-concentration capsaicin patch and experience in its use in the management of neuropathic pain – 2013
  2. Use of complementary and alternative medicine by patients with hypermobile Ehlers–Danlos Syndrome- A qualitative study – 2022
  3. Multidisciplinary Approach to Treating Chronic Pain in Patients with Ehlers–Danlos Syndrome- Critically Appraised Topic – 2022