2017 Criteria (Hypermobile Ehlers-Danlos)
Applies the 2017 international criteria for hypermobile Ehlers-Danlos syndrome.
Open the calculator → Runs in your browser. Data leaves only if you deliberately send a problem report from the interactive tool.
Example
- Beighton score out of 9
- 6
- Which Beighton cutoff applies
- Pubertal, up to age 50 (cutoff 5)
- Soft or velvety skin
- Yes
- Mild skin hyperextensibility
- Yes
- Unexplained striae
- Yes
- Piezogenic heel papules
- Yes
- Recurrent abdominal hernias
- Yes
- First-degree relative meets the criteria
- Yes
- No unusual skin fragility
- Yes
- Other connective-tissue disorders excluded
- Yes
- Alternative causes excluded
- Yes
Result: criteria for hypermobile Ehlers-Danlos syndrome are met
The tool opens with these values already filled in. Replace them with your own.
What the result means
- All three
- Criteria 1, 2 and 3 must hold at the same time. Any one of them failing means the criteria are not met.
- Criterion 1
- Beighton cutoff by group: 6 or more for pre-pubertal children and adolescents, 5 or more to age 50, 4 or more above 50.
- One point below
- At exactly one point below the cutoff, two or more of the five-part questionnaire items also meet criterion 1. Two points below is not rescued.
- Criterion 2
- Two or more of: feature A, five of twelve connective-tissue findings; feature B, a first-degree relative meeting the criteria; feature C, one of three musculoskeletal complications.
- Criterion 3
- All three: no unusual skin fragility; other heritable and acquired connective-tissue disorders excluded; alternative causes of hypermobility excluded.
- The acquired-CTD rule
- In lupus, rheumatoid arthritis or another acquired connective-tissue disorder, criterion 2 needs BOTH features A and B, and feature C cannot be counted at all.
- Why that matters
- It bites in the group most often assessed. Chronic pain and instability are least specific there, so "any two of A, B and C" would diagnose hEDS where the criteria forbid it.
What you enter
- Beighton score out of 9
- Which Beighton cutoff applies
- Hands flat on the floor
- Thumb to forearm
- Contorted body as a child
- Shoulder or kneecap dislocated twice
- Considers self double-jointed
- Soft or velvety skin
- Mild skin hyperextensibility
- Unexplained striae
- Piezogenic heel papules
The other 16 fields
- Recurrent abdominal hernias
- Atrophic scarring, 2+ sites
- Pelvic, rectal or uterine prolapse
- Dental crowding, high palate
- Arachnodactyly, bilateral
- Arm span to height 1.05 or more
- Mitral valve prolapse
- Aortic root Z above 2
- First-degree relative meets the criteria
- Limb pain daily for 3 months
- Chronic widespread pain
- Recurrent dislocations or instability
- No unusual skin fragility
- Other connective-tissue disorders excluded
- Alternative causes excluded
- Patient has an acquired connective-tissue disorder
How this is calculated
Citation
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. Read the source ↗
A reference and educational tool. Not medical, legal, or financial advice, and not a substitute for clinician judgment.