The diagnosis of hEDS is mainly a clinical one, based on the Beighton Score and other criteria that are described below.
9 points in total.
Can you now or could you in the past:
Bend the thumbs backwards and it touches your forearm (1 point for each side)
Bend the little fingers backwards beyond 90 degrees (1 point for each side)
Bend the elbows backwards more than 10 degrees (1 point for each side)
Bend the knees backwards more than 10 degrees (1 point for each side)
Place your palms on the ground with the legs straight while standing and bending forward (1 point)
New classification of hypermobile Ehlers-Danlos snydrome 2017
MALFAIT, Fransiska, et al. The 2017 international classification of the Ehlers–Danlos syndromes. In: American Journal of Medical Genetics Part C: Seminars in Medical Genetics. 2017. S. 8-26.
Hypermobility is classified as:
- Localized joint hypermobility: hypermobility in less than five joints
- Generalized joint hypermobility: hypermobility in more than five joints
- Peripheral joint hypermobility: hypermobility in hands or feet only
- Historical joint hypermobility: hypermobility in the past; five point questionaire
- Joint instability
Furthermore it is divided into:
- People with asymptomatic hypermobility without other medical conditions
- People that are symptomatic and hypermobile but do not meet the new hEDS criteria. The new term is: Hypermobility spectrum disorder (HSD)
Hypermobility Spectrum Disorders are further divided into:
- Localized Hypermobility Spectrum Disorder (L-HSD)
- Peripheral Hypermobility Spectrum Disorder (P-HSD)
- Generalized Hypermobility Spectrum Disorder (G-HSD)
- Historical Hypermobility Spectrum Disorder (H-HSD)
New criteria for hEDS:
The hypermobile EDS patient must now fulfill three different criteria. Criterium 2 is additionally divided into Feature A – C.
Positive Beighton Score
Children: more than 6 out of 9
People older than 50 years: More than 4 out of 9
People younger than 50 years: More than 5 out of 9
To fulfill criterium 2, two or more of the below mentioned features must be present.
*Skin is hyperextensible if it can be stretched over a cut off in three of the following areas: > 1.5 cm for the dorsum of the hands and distal part of the forearms; > 3 cm for neck, elbow and knees.
All of the below mentioned must be present:
- Exclusion of other connective tissue disorders
- Absence of unusual skin fragility
- Absence of alternative diagnoses that include hypermobility and hypotonia such as myopathy