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Diagnosis and the 2017 hEDS Criteria

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Hypermobile Ehlers-Danlos Syndrome (hEDS) is diagnosed clinically using the strict 2017 criteria, as there is no genetic test. Diagnosis requires meeting three criteria: generalized joint hypermobility (measured by the Beighton score), systemic signs or family history, and the exclusion of other conditions.

Key Takeaways

  • • hEDS is the most common type of Ehlers-Danlos Syndrome and the only type that cannot currently be diagnosed with a genetic test.
  • • Doctors use the 2017 clinical criteria, which includes the 9-point Beighton score, to evaluate joint hypermobility.
  • • To be diagnosed with hEDS, you must meet criteria for joint hypermobility, systemic issues or family history, and rule out other conditions.
  • • Patients who have painful hypermobile joints but do not meet the full hEDS criteria may be diagnosed with Hypermobility Spectrum Disorder (HSD).
  • • HSD is not a milder condition than hEDS; patients experience similar levels of pain and require the same physical therapy and care.

The way doctors diagnose Ehlers-Danlos Syndrome (EDS) changed significantly in 2017. For most types of EDS, a diagnosis is confirmed with a genetic test [1]. However, for Hypermobile EDS (hEDS)—the most common type—there is no known genetic marker yet [2]. This means hEDS is a clinical diagnosis, based entirely on a physical exam and your medical history [3].

If you do not meet the strict 2017 requirements for hEDS but still have symptomatic joint hypermobility, you may be diagnosed with Hypermobility Spectrum Disorder (HSD) [4][5].

The 3 Criteria for hEDS

To be diagnosed with hEDS, a patient must meet all three of the following criteria simultaneously [3][6]:

Criterion 1: Generalized Joint Hypermobility (GJH)

Doctors use the Beighton Score, a 9-point system, to measure how flexible your joints are [7]. Warning: Do not forcefully push your own joints into these unnatural positions at home to “test” yourself, as this can easily cause subluxations or dislocations.

You get one point for each of the following [8]:

  • Bending your pinky finger back past 90 degrees (Left and Right)
  • Pushing your thumb down to touch your forearm (Left and Right)
  • Hyperextending your elbows past 10 degrees (bending slightly backward past a straight line) (Left and Right)
  • Hyperextending your knees past 10 degrees (bending slightly backward past a straight line) (Left and Right)
  • Placing your palms flat on the floor while keeping your legs straight (1 point)

Because we naturally become less flexible as we get older, the “passing” score changes with age [9][10]:

  • Pre-pubertal children: ≥6/9\ge 6/9
  • Pubertal adolescents and adults up to age 50: ≥5/9\ge 5/9
  • Adults (over age 50): ≥4/9\ge 4/9

Criterion 2: Systemic Signs and Family History

This criterion ensures the condition affects more than just your joints. You must meet two or more of these categories [11][9]:

  • Feature A (Systemic Signs): Having at least 5 out of 12 specific physical traits, such as very soft skin, unexplained stretch marks, dental crowding, or a certain type of heart valve issue (mitral valve prolapse) [11].
  • Feature B (Family History): Having a parent, sibling, or child who independently meets the 2017 hEDS criteria [11].
  • Feature C (Musculoskeletal Issues): Having daily musculoskeletal pain in two or more limbs for over 3 months, or chronic widespread pain, or having recurring joint dislocations/instability [11].

Criterion 3: Excluding Other Conditions

Your doctor must confirm that your symptoms aren’t better explained by other things, like autoimmune diseases (e.g., Lupus) or other types of EDS [3][11].

When is Genetic Testing Needed?

While hEDS has no genetic test, your doctor may order testing if they see “red flags” for other types of EDS [3][12].

  • Vascular EDS (vEDS) Red Flags: Unexplained organ or artery rupture, very thin/translucent skin, or a family history of sudden vascular events [13][14].
  • Classical EDS (cEDS) Red Flags: Extremely stretchy skin and “cigarette paper” scars that are wide and thin [15][16].

hEDS vs. Hypermobility Spectrum Disorder (HSD)

If you have painful, unstable joints but don’t meet the “systemic” requirements (Criterion 2, Feature A), you will likely be diagnosed with HSD [4][5]. It is important to know that HSD is not “EDS-lite.” Patients with HSD often face the same levels of pain, fatigue, and disability as those with hEDS and deserve the same level of care and physical therapy [5][17]. In some cases, as a person gets older and develops more symptoms, their diagnosis may shift from HSD to hEDS [9][18].

Frequently Asked Questions

Is there a genetic test for hypermobile EDS (hEDS)?
No, there is currently no known genetic marker for hypermobile EDS. It is diagnosed clinically by a doctor based on your physical exam, medical history, and the strict 2017 diagnostic criteria.
What is the Beighton Score?
The Beighton Score is a 9-point system doctors use to measure generalized joint flexibility. It tests specific joints, like the knees, elbows, thumbs, and pinky fingers, to see if they bend backward beyond normal limits.
What is the difference between hEDS and HSD?
Hypermobility Spectrum Disorder (HSD) is diagnosed when you have symptomatic joint hypermobility but do not meet the full systemic criteria for hEDS. Both conditions can cause similar levels of pain and disability and require similar care.
Will I need a genetic test if I am evaluated for EDS?
While hEDS cannot be diagnosed with a genetic test, your doctor may order one if they see warning signs for other rarer types of Ehlers-Danlos Syndrome, such as the Vascular or Classical types.

Questions for Your Doctor

  • • Can we walk through the three criteria for hEDS together to see which ones I meet?
  • • What is my Beighton score, and how does my age affect the cutoff you are using?
  • • If I don't meet the full hEDS criteria, do I meet the criteria for Hypermobility Spectrum Disorder (HSD)?
  • • Are there any 'red flags' in my history that suggest we should do genetic testing for other EDS types, like the Vascular or Classical types?
  • • How will my treatment plan differ if my diagnosis is HSD versus hEDS?

Questions for You

  • • Can I place my palms flat on the floor without bending my knees? Have I been able to do this in the past?
  • • Do I have any first-degree relatives (parents, siblings, children) who have been formally diagnosed with hEDS using the 2017 criteria?
  • • Do I experience daily joint pain in two or more limbs that has lasted for more than three months?
  • • Have I had more than one joint dislocation in the same joint, or dislocations in two different joints, without a major injury?

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References

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This page explains the 2017 hEDS diagnostic criteria for educational purposes. Only a qualified healthcare provider can perform a clinical exam, calculate a Beighton score, and formally diagnose Ehlers-Danlos Syndrome or HSD.

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