hEDS vs HSD: What Is the Difference?
Flexible joints that cause pain, dislocations, or fatigue often lead to one of two labels: hypermobile Ehlers-Danlos syndrome (hEDS) and hypermobility spectrum disorder (HSD). The two overlap so much that the line between them confuses patients and clinicians alike.
The short version
hEDS and HSD look very similar. The difference is how strictly a person meets the formal checklist, not how sick they are.
How hEDS is diagnosed
hEDS is diagnosed when a person meets all three criteria:
Generalized joint hypermobility, measured by the Beighton score.
Two or more of the following: at least 5 of 12 specific physical signs (for example soft skin, unexplained stretch marks, piezogenic heel papules, dental crowding, mitral valve prolapse, long fingers); a first-degree relative who independently meets hEDS criteria; musculoskeletal complications such as chronic pain, recurrent dislocations, or joint instability.
Other diagnoses are ruled out, including other EDS types, other connective tissue disorders, lupus, and rheumatoid arthritis.
What is HSD?
HSD is the term for symptomatic joint hypermobility, flexible joints that cause real problems like pain, dislocations, or fatigue, in a person who does not meet the full hEDS criteria.
People with HSD often have many of the same comorbidities seen in hEDS, including POTS and other forms of dysautonomia, functional gastrointestinal problems, migraine, fatigue, and anxiety.
What to understand about the two labels
HSD is not a lesser diagnosis. People with HSD can be just as symptomatic and disabled as people with hEDS. Both groups show similar rates of chronic pain, fatigue, digestive problems, and autonomic symptoms such as dizziness on standing.
The split is based on a checklist, not severity. Many clinicians view hEDS and HSD as parts of one spectrum rather than two separate conditions.
Treatment is the same for both: physical therapy, pain management, joint protection, psychological support, and care for associated symptoms.
Genetic testing: what it can and cannot do
For the genetically defined subtypes (every type except hEDS)
Testing is very useful and often essential.
It confirms the subtype. Every subtype except hEDS has a known genetic cause, so a multigene panel can identify which one a person has.
The subtype changes management. Confirmed vascular EDS means screening for arterial aneurysms. Kyphoscoliotic EDS means monitoring the spine and eyes.
It guides family planning. A confirmed diagnosis supports accurate genetic counseling for relatives and future pregnancies.
Diagnostic yield is high. In patients who meet clinical criteria for a specific subtype, testing confirms the diagnosis in most cases. In one small pediatric cohort, most children who met criteria were confirmed (Damseh et al., 2022).
For hEDS and HSD
What testing cannot do:
It cannot confirm hEDS or HSD. No causative gene is known, so the diagnosis is clinical.
A negative test does not rule out hEDS or HSD, because the responsible gene or genes have not been found.
It cannot tell hEDS and HSD apart.
What testing can do:
Rule out look-alike conditions. This is the main reason to test. In patients who clinically appear to have hEDS, testing can reveal a different or additional diagnosis that needs different management. In one study, about 26.4% of clinically suspected hEDS patients had an alternative or additional diagnosis on genetic testing (Forghani et al., 2025).
Testing reduces the likelihood of higher-risk connective tissue disorders that can carry serious or life-threatening complications.
Support family planning by ruling out other inherited conditions.
Contribute to research that may eventually identify the gene or genes behind hEDS/HSD.
Bottom line: For suspected hEDS or HSD, genetic testing is used to make sure nothing more serious is being missed, not to confirm the diagnosis. A normal result does not mean there is no condition. hEDS and HSD stay clinical diagnoses.
Practical notes
If hEDS or HSD runs in the family, genetic counseling and testing can help explain personal health implications, recurrence information, and relevant genetic testing options, keeping in mind there is no confirmatory test for hEDS itself.
hEDS and HSD are lifelong. There is no cure, but symptoms can be managed with the right care team.
Dentists, surgeons, and anesthesiologists should be told about a hEDS or HSD diagnosis. Many people with hEDS/HSD have a reduced response to local anesthetics, and hypermobile joints need careful positioning during procedures to lower the risk of dislocation.
Research into the causes of hEDS and HSD is ongoing.
What may change in 2026
The way hEDS and HSD are defined is under active review. The Ehlers-Danlos Society is running its Road to 2026 initiative to update the EDS classification and diagnostic criteria, with revised recommendations expected in late 2026. A proposed new model is being tested in real-world clinics, and findings from large genetic and biomarker studies, such as the HEDGE study, may eventually feed into the criteria.
What this means in practice: the current line between hEDS and HSD could shift. The two are defined separately today, but many researchers already treat them as one spectrum, and a revised framework may redraw or remove that division. A current hEDS or HSD diagnosis could carry a different label under a future update. That would not change the underlying symptoms or the care required. This page will be updated as the new criteria are published.
Learn more or get evaluated
New to this? Start with the guide to the types of Ehlers-Danlos syndromes.
Anyone wondering whether hEDS or HSD could explain their symptoms can book an hEDS evaluation.
Sources: Malfait F, et al. The 2017 international classification of the Ehlers-Danlos syndromes. Am J Med Genet C. 2017. | Damseh N, et al. Diagnostic outcomes for molecular genetic testing in children with suspected Ehlers-Danlos syndrome. Am J Med Genet A. 2022. | Forghani I, See J, McGonigle WC. Hypermobile Ehlers-Danlos syndrome: diagnostic challenges and the role of genetic testing. Genes. 2025.
