hEDS diagnostic criteria checker

Which group applies to you?

Your Beighton score (0–9)

Don't know it? Work through the nine manoeuvres with illustrations, then come back. Your score will be offered here automatically.

Unusually soft or velvety skin

Mild skin hyperextensibility (skin that stretches further than usual, then returns)

Unexplained stretch marks (striae distensae or rubrae) on the back, groins, thighs, breasts and/or abdomen in adolescents, men, or pre-pubertal women, without a history of significant gain or loss of body fat or weight

Bilateral piezogenic papules of the heel (small soft lumps on both heels that appear when standing)

Recurrent or multiple abdominal hernias

Atrophic scarring at two or more sites, without the papery or dark-stained scars seen in classical EDS clinician / imaging

Pelvic floor, rectal, and/or uterine prolapse in children, men, or women who have not given birth, without a history of morbid obesity or another known predisposing condition

Dental crowding and a high or narrow palate

Arachnodactyly: a positive wrist sign (Walker sign) on both sides, or a positive thumb sign (Steinberg sign) on both sides

Arm span-to-height ratio of 1.05 or more

Mitral valve prolapse, mild or greater, on strict echocardiographic criteria clinician / imaging

Aortic root dilatation with a Z-score above +2 clinician / imaging

Do you have one or more first-degree relatives (parent, sibling, or child) who independently meets the current criteria for hEDS?

A relative who is "bendy" or has an older hypermobility diagnosis does not count unless they meet the 2017 criteria in their own right.

Absence of unusual skin fragility (which should prompt consideration of other types of EDS)

Exclusion of other heritable and acquired connective tissue disorders, including autoimmune rheumatological conditions

Exclusion of alternative diagnoses that may also involve joint hypermobility through low muscle tone or connective tissue laxity, such as neuromuscular disorders (e.g. Bethlem myopathy), other hereditary connective tissue disorders (other EDS types, Loeys-Dietz syndrome, Marfan syndrome), and skeletal dysplasias (e.g. osteogenesis imperfecta)

Have you been diagnosed with an acquired connective tissue disorder, such as lupus or rheumatoid arthritis?

If so, the criteria change: Feature C (pain and instability) cannot be counted, and both Feature A and Feature B are required.