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
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
The other 17 fields
  • Piezogenic heel papules
  • 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

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.

Browse all tools

Built by Clay Good. Source on GitHub.