Hypermobile Ehlers Danlos Syndrome

Diagnostic Criteria

Criterion 1: Generalised Joint Hypermobility

Must meet Beighton Score for age

Age	Beighton Score
Prepubescent or adolescent	≥6
Pubescent up until age 50	≥5
Over age 50	≥4
Patients with Acquired joint limitations	BS 1 point under age requirements AND a positive 5PQ

Criterion 2: At least 2 features must be present

Feature A:Systemic manifestations of CTD (need ≥5)'

1. Unusually soft/velvety skin
2. Mild skin hyperextensibility
3. Unexplained striaedistensae/rubrae
4. Bilateral piezogenic papules of heel
5. Recurrent/multiple abdominal hernia
6. Atrophic scaring in ≥2 sites
7. Pelvic floor, rectal, and/or uterine prolapse in children, men or nulliparous women
8. Dental crowding and high or narrow palate
9. Arachnodactyly
10. Arm span-to-height ≥1.05
11. Mitral valve prolapse
12. Aortic root dilatation with Z score > +2

Feature B: Family history (1 or more first-degree relatives must meet criteria)

Feature C: MSK Complications(need ≥1)

1. MSK pain in ≥2 limbs, recurring daily for ≥3 months
2. Chronic widespread pain for ≥3 months
3. Recurrent joint dislocations or frank joint instability, in the absence of trauma (a or b)
   • ≥3 atraumatic dislocations in same joint or ≥2 more atraumatic dislocations in two difference joints occurring at different times
   • Medical confirmation of joint instability at two or more sites not related to trauma

Criterion 3: All three prerequisites must be met

1. Absence of unusual skin fragility.
2. Exclusion of other heritable and acquired connective tissue disorders. In patients with an acquired onnective tissue disorder, additional diagnosis of hEDS requires meeting both Features A and B of Criterion 2. Feature C of Criterion 2 cannot be counted in this situation.
3. Exclusion of alternative diagnoses that may also include joint hypermobility by means of hypotonia and/or connective tissue laxity