Hypermobile Ehlers-Danlos Syndrome (hEDS)

Being “bendy” isn’t a diagnosis, and many hypermobile people never have problems ^[8]. But if your joints slip out of place, you hurt every day and nobody can explain why, hEDS or a related condition may be part of the picture. This page explains what hEDS is, how doctors diagnose it, and what usually helps.

What hEDS is

The Ehlers-Danlos syndromes are a group of 13 heritable connective tissue disorders. Each type has its own features and diagnostic criteria ^{[2, 9]}. For most types, a genetic test confirms the diagnosis. For hEDS, the genetic cause hasn’t been found, so diagnosis rests on clinical findings ^{[2, 9]}.

hEDS appears to follow an autosomal dominant inheritance pattern, which means a person with hEDS has a 50% chance of passing it on to each child ^[1].

How common is it? The Ehlers-Danlos Society says hEDS is thought to affect at least 1 in 3,100–5,000 people, but the true number isn’t known and may be underestimated ^[1]. A national study of health records in Wales found a diagnosed prevalence of EDS or joint hypermobility syndrome of about 194 per 100,000 people (roughly 1 in 500), with 70% of those diagnosed being women ^[10].

hEDS vs. HSD. The 2017 framework also created hypermobility spectrum disorders (HSD) for people with symptomatic joint hypermobility who don’t meet the criteria for hEDS or another syndrome ^[11]. HSD can cause the same kinds of problems, such as joint instability, injury, pain, fatigue, headaches, gut problems and autonomic dysfunction, and symptoms can range from mild to severe ^[8]. Some people diagnosed with “EDS hypermobility type” before 2017 may now fit HSD instead ^[7].

Common symptoms

According to the Ehlers-Danlos Society, hEDS may be suspected in someone with ^[1]:

• Joint hypermobility
• Joint instability (joints that slip partly out of place, called subluxations, or dislocate)
• Chronic pain
• Mildly stretchy skin
• Abnormal scarring

People with hEDS may also have chronic fatigue, gut problems, dysautonomia (problems with the automatic nervous system, such as POTS), headaches and mast cell activation diseases ^[1].

Pain is very common. In one study, nearly 90% of people with EDS reported some form of chronic pain. It can come from subluxations and dislocations, past surgery, muscle weakness, poor joint position sense (proprioception) and spinal instability ^[5].

The “hidden” symptoms. The 2017 criteria note that features such as sleep problems, fatigue, POTS, functional gut disorders, dysautonomia, anxiety and depression are often seen in hEDS. They aren’t in the criteria because they aren’t specific enough, but they “may be more debilitating than the joint symptoms” and should always be asked about ^[2].

Causes and risk factors

• Genetics: hEDS runs in families in an autosomal dominant pattern, but the responsible gene(s) are unknown ^{[1, 2]}.
• Sex: more women than men are diagnosed ^[10].
• Hypermobility alone isn’t hEDS. Up to about 20% of adults remain hypermobile, and hypermobility that doesn’t cause pain or other symptoms doesn’t need treatment ^[8].

How hEDS is diagnosed

There’s no lab test for hEDS. Doctors use the 2017 diagnostic criteria, and a person must meet all three parts ^{[2, 3]}:

Criterion 1: Generalized joint hypermobility, usually measured with the 9-point Beighton score. The cut-off depends on age: at least 6 for children and adolescents before puberty, at least 5 from puberty to age 50, and at least 4 over age 50. A short questionnaire can help if the score is one point short ^[3].

Criterion 2: At least two of these three features ^[3]:

• Feature A: at least 5 of 12 signs of a body-wide connective tissue disorder, such as unusually soft or velvety skin, mildly stretchy skin, unexplained stretch marks, small heel bumps when standing (piezogenic papules), hernias, atrophic scars, pelvic organ prolapse in certain groups, dental crowding and high or narrow palate, long fingers (arachnodactyly), an arm span long relative to height, mitral valve prolapse, or a mildly widened aortic root.
• Feature B: a first-degree relative (parent, sibling or child) who independently meets the hEDS criteria.
• Feature C: at least one musculoskeletal complication: pain in two or more limbs daily for at least 3 months, widespread pain for at least 3 months, or recurrent dislocations or joint instability without injury.

Criterion 3: Rule-outs. No unusual skin fragility (which points to other EDS types), and other conditions that cause hypermobility have been excluded. These include other heritable connective tissue disorders (such as other EDS types, Marfan or Loeys-Dietz syndrome), autoimmune rheumatic conditions, neuromuscular disorders and skeletal dysplasias ^[3].

Who diagnoses it? The checklist is designed for doctors across all specialties ^[3]. Your primary care doctor can help you decide whether to see a specialist such as a rheumatologist or geneticist. Genetic testing may be used to rule out other types, especially if there are signs of a more serious type such as vascular EDS ^[2].

Children. Children and teens with joint hypermobility can be assessed using a separate pediatric framework ^[1].

Treatment approaches to discuss with your clinician

There’s no cure, and research on specific treatments is limited ^{[4, 5]}. Care usually focuses on protecting joints, reducing pain and supporting function, often with several providers ^[1].

• Physical therapy plays a central role. The aim is usually to build strength, stability, joint position sense and endurance in a way that doesn’t cause injury. The evidence base is still small and more trials are needed ^[4].
• Pain management may combine treating the cause of pain (for example, a joint dislocation) with strategies to reduce pain itself. Options include physical therapy, medicines, and equipment such as cushions, compression garments and braces ^[5].
• Occupational therapy, splints and mobility aids are among the management topics covered by the Ehlers-Danlos Society ^[1].
• Gut symptoms are common and often meet criteria for functional gut disorders such as irritable bowel syndrome. They’re usually treated based on symptoms, since specific guidelines don’t yet exist ^[12].
• Autonomic symptoms such as POTS are usually managed first with education, avoiding triggers, building fitness and staying hydrated. Medicines are considered when daily function is moderately to severely affected ^[6].
• Surgery and procedures: let your surgical team know you have hEDS, since certain precautions may be needed ^[12].

Living with hEDS: practical day-to-day help

• Pace activity. Build up gradually with a physical therapist who understands hypermobility rather than pushing through pain ^[4].
• Protect your joints with supportive equipment where it helps, such as braces, ring splints or cushions ^{[1, 5]}.
• Track your “whole body” symptoms like fatigue, sleep, gut and dizziness, not just joints, because they often matter as much as the joints ^[2].
• Take your mental health seriously. Anxiety and depression are commonly reported in hEDS ^[2]. Support helps, and if you’re in crisis, call or text 988 in the U.S. ^[13].
• Find community. The Ehlers-Danlos Society offers education, support and a directory for finding knowledgeable doctors and therapists (see Helpful resources below) ^[1].
• See our overlap post and Trifecta reference.

How hEDS overlaps with the other conditions

• POTS. In one study of 91 people with POTS, 31% met the hEDS criteria and another 24% had generalized joint hypermobility ^[14]. Specialist clinics report that autonomic problems are frequently seen in hEDS, though exact rates haven’t been measured ^[6].
• MCAS. The Ehlers-Danlos Society lists mast cell activation diseases among conditions people with hEDS may have ^[1], and some researchers have proposed a role for mast cells in EDS ^[15].
• The “trifecta” debate. A 2020 review by Kucharik and Chang looked for research linking hEDS, POTS and MCAS. It found few original studies, no shared mechanism yet described, and concluded that current evidence doesn’t establish a relationship, partly because the conditions share vague, overlapping symptoms ^[7]. Other researchers report a strong clinical overlap and argue strict definitions can miss people who’d benefit from treatment ^{[14, 16]}. Both views are worth knowing.
• Mold toxicity and CIRS. We didn’t find research showing that mold causes or worsens hEDS. Some people with hEDS also live with mold-related illness, but that’s not the same as a proven link.

What’s known and what’s debated

Generally accepted: hEDS is a heritable connective tissue disorder diagnosed with the 2017 clinical criteria; physical therapy and pain management are core parts of care ^{[1, 2, 4]}.

Still debated or unknown:

• The genetic cause ^[2].
• How common hEDS really is, since records show more diagnoses than older estimates suggested ^{[1, 10]}.
• Where to draw the line between hEDS and HSD ^{[7, 11]}.
• Whether, and how, hEDS is biologically linked to POTS and MCAS ^{[6, 7, 15]}.
• Which treatments work best. High-quality trials are lacking ^{[4, 5]}.

Want to know who’s behind this site? Read our story.

What to do next

General information, not a diagnosis or a treatment plan. A licensed clinician can tell you which of these steps fit you.

1. Step 1Start this week

   • Track whole-body symptoms like fatigue, sleep, gut problems and dizziness, not just joints ^[2]. AHRQ suggests writing down all your questions and concerns before a visit ^[19].
   • Note whether a parent, sibling or child has similar features, since family history is part of the diagnostic criteria ^[3].
   • Our symptom checklist can help you organize what you notice.

2. Step 2Choose who to see

   • Start with your primary care doctor: AHRQ calls your doctor “the first person to ask” ^[19].
   • In the UK’s NHS, a GP may refer people with joint problems to a rheumatologist, and to a genetics service if a rarer EDS type is possible ^[20]. People with hEDS often need several providers in different specialties ^[1].
   • The Ehlers-Danlos Society keeps a directory of healthcare professionals, and it notes that it doesn’t certify the providers listed ^[21].
   • See also the provider directories on our homepage.

3. Step 3Tests to ask about

   • There’s no lab test for hEDS, so ask whether you meet the 2017 diagnostic criteria ^{[1, 3]}.
   • The Ehlers-Danlos Society’s criteria checklist is designed for doctors in all specialties ^[3].
   • Genetic testing may be used to rule out other EDS types ^[2].

4. Step 4Everyday steps mainstream sources support

   • Physical therapy plays a central role in building strength and stability without injury ^[4].
   • Protect your joints with braces, ring splints or cushions where they help ^{[1, 5]}, and build up activity gradually ^[4].
   • For dizziness or POTS-type symptoms, care usually starts with education, avoiding triggers, building fitness and staying hydrated ^[6].

5. Step 5Prepare for the appointment

   • Bring a list of all the medicines you take ^[19]. Our Doctor visit prep sheet has space for your joint history and past results.
   • If you feel dismissed, consider a second opinion: AHRQ says wanting one “is not pushy or rude” and suggests asking for a doctor who doesn’t work in the same office ^[19].
   • The Ehlers-Danlos Society offers education and support ^[1].
   • Then use the Questions to ask your doctor below.

6. Step 6When to get urgent care

   • Call 911 for a suspected dislocation or broken bone, or a limb that’s pale, cold or blue below an injured joint ^[17], or if someone faints and doesn’t become alert within a couple of minutes ^[18]. If you’re having thoughts of suicide, call or text 988 ^[13].
   • See the full list under When to get urgent help.

Questions to ask your doctor

• Do I meet the 2017 hEDS criteria, or is HSD a better fit?
• Should we rule out other connective tissue disorders, including with genetic testing?
• Can you refer me to a physical therapist experienced with hypermobility?
• What’s our plan for managing pain and preventing injuries?
• Should I be checked for POTS or other autonomic problems, or for mast cell problems?
• What should I tell surgeons, dentists or anesthesiologists before a procedure?

Sources

Numbers in brackets in the text link to these sources. Links were checked in October 2026. Some sources are written by people who support a particular approach; we've tried to say so in the text.

1. The Ehlers-Danlos Society. Hypermobile Ehlers-Danlos Syndrome (hEDS). Web page, accessed October 10, 2026. https://www.ehlers-danlos.com/heds/
2. Malfait F, Francomano C, Byers P, et al. The 2017 international classification of the Ehlers-Danlos syndromes. Am J Med Genet C Semin Med Genet. 2017;175(1):8-26. PMID: 28306229. https://doi.org/10.1002/ajmg.c.31552
3. The Ehlers-Danlos Society. Diagnostic Criteria for Hypermobile Ehlers-Danlos Syndrome (hEDS) checklist. Web page, accessed October 10, 2026. https://www.ehlers-danlos.com/wp-content/uploads/2017/05/hEDS-Dx-Criteria-checklist-1.pdf
4. Engelbert RH, Juul-Kristensen B, Pacey V, et al. The evidence-based rationale for physical therapy treatment of children, adolescents, and adults diagnosed with joint hypermobility syndrome/hypermobile Ehlers Danlos syndrome. Am J Med Genet C Semin Med Genet. 2017;175(1):158-167. PMID: 28306230. https://doi.org/10.1002/ajmg.c.31545
5. Chopra P, Tinkle B, Hamonet C, et al. Pain management in the Ehlers-Danlos syndromes. Am J Med Genet C Semin Med Genet. 2017;175(1):212-219. PMID: 28186390. https://doi.org/10.1002/ajmg.c.31554
6. Hakim A, O'Callaghan C, De Wandele I, et al. Cardiovascular autonomic dysfunction in Ehlers-Danlos syndrome-Hypermobile type. Am J Med Genet C Semin Med Genet. 2017;175(1):168-174. PMID: 28160388. https://doi.org/10.1002/ajmg.c.31543
7. Kucharik AH, Chang C. The Relationship Between Hypermobile Ehlers-Danlos Syndrome (hEDS), Postural Orthostatic Tachycardia Syndrome (POTS), and Mast Cell Activation Syndrome (MCAS). Clin Rev Allergy Immunol. 2020;58(3):273-297. PMID: 31267471. https://doi.org/10.1007/s12016-019-08755-8
8. The Ehlers-Danlos Society. What is HSD?. Web page, accessed October 10, 2026. https://www.ehlers-danlos.com/what-is-hsd/
9. The Ehlers-Danlos Society. What is EDS?. Web page, accessed October 10, 2026. https://www.ehlers-danlos.com/what-is-eds/
10. Demmler JC, Atkinson MD, Reinhold EJ, et al. Diagnosed prevalence of Ehlers-Danlos syndrome and hypermobility spectrum disorder in Wales, UK: a national electronic cohort study and case-control comparison. BMJ Open. 2019;9(11):e031365. PMID: 31685485. https://pmc.ncbi.nlm.nih.gov/articles/PMC6858200/
11. Castori M, Tinkle B, Levy H, Grahame R, Malfait F, Hakim A. A framework for the classification of joint hypermobility and related conditions. Am J Med Genet C Semin Med Genet. 2017;175(1):148-157. PMID: 28145606. https://doi.org/10.1002/ajmg.c.31539
12. Fikree A, Chelimsky G, Collins H, Kovacic K, Aziz Q. Gastrointestinal involvement in the Ehlers-Danlos syndromes. Am J Med Genet C Semin Med Genet. 2017;175(1):181-187. PMID: 28186368. https://doi.org/10.1002/ajmg.c.31546
13. National Institute of Mental Health (NIMH). Help for Mental Illnesses. Web page, accessed October 10, 2026. https://www.nimh.nih.gov/health/find-help
14. Miller AJ, Stiles LE, Sheehan T, et al. Prevalence of hypermobile Ehlers-Danlos syndrome in postural orthostatic tachycardia syndrome. Auton Neurosci. 2020;224:102637. PMID: 31954224. https://pmc.ncbi.nlm.nih.gov/articles/PMC7282488/
15. Seneviratne SL, Maitland A, Afrin L. Mast cell disorders in Ehlers-Danlos syndrome. Am J Med Genet C Semin Med Genet. 2017;175(1):226-236. PMID: 28261938. https://doi.org/10.1002/ajmg.c.31555
16. Yao L, Subramaniam K, Raja KM, et al. Association of postural orthostatic tachycardia syndrome, hypermobility spectrum disorders, and mast cell activation syndrome in young patients; prevalence, overlap and response to therapy depends on the definition. Front Neurol. 2025;16:1513199. PMID: 40352770. https://pmc.ncbi.nlm.nih.gov/articles/PMC12063504/
17. MedlinePlus. Dislocation. Web page, accessed October 10, 2026. https://medlineplus.gov/ency/article/000014.htm
18. MedlinePlus. Fainting. Web page, accessed October 10, 2026. https://medlineplus.gov/ency/article/003092.htm
19. Agency for Healthcare Research and Quality (AHRQ). Next Steps After Your Diagnosis: Finding Information and Support (patient booklet, PDF). Accessed October 17, 2026. https://www.ahrq.gov/sites/default/files/wysiwyg/patients-consumers/diagnosis-treatment/diagnosis/diaginfo/diaginfo.pdf
20. NHS (UK). Ehlers-Danlos syndromes. Page last reviewed October 4, 2022. Web page, accessed October 17, 2026. https://www.nhs.uk/conditions/ehlers-danlos-syndromes/
21. The Ehlers-Danlos Society. Healthcare Professionals Directory. Web page, accessed October 17, 2026. https://www.ehlers-danlos.com/healthcare-professionals-directory/