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. 2022 Jul 20;37(9):1693–1709. doi: 10.1111/jgh.15927

Table 1.

New diagnostic criteria for hypermobile Ehlers–Danlos syndrome (hEDS) 7

Criterion 1:

Must be met

The Beighton score for generalized joint hypermobility

  • Prepubertal children/adolescents: > 6

  • Men, women post puberty to age 50: > 5

  • Over age 50: > 4

  • If one point below diagnostic, score ≥ 2 in the 5‐point questionnaire indicates the presence of joint hypermobility (sensitivity 80–85%, specificity 80–90%) 10

Criterion 2:

Two or more of the following features

Feature A:

Systemic manifestations of a more generalized connective tissue disorder

 At least five of the following must be present:

  • Unusually soft or velvety skin

  • Mild skin hyperextensibility (skin stretch > 1.5 cm distal forearm/dorsum hand; > 3 cm neck/elbow/knee; > 1 cm palmar surface hand)

  • Unexplained striae (without a history of significant weight change)

  • Bilateral piezogenic papules of heel (small, tender herniations of adipose globules through fascia into dermis)

  • Recurrent or multiple abdominal hernias

  • Atrophic scarring involving at least two sites

  • Pelvic floor, rectal and/or uterine prolapse in children, men or nulliparous women without a history of morbid obesity or predisposing medical condition

  • Dental crowding and high or narrow palate

  • Arachnodactyly

  • Arm span‐to‐height ratio ≥ 1.05

  • Mitral valve prolapse (based on strict echocardiographic criteria)

  • Aortic root dilatation with z‐score > +2 (i.e. > 2 SD above the size and gender specific population mean)

Feature B:

Positive family history

  • ≥ 1 first‐degree relative with hEDS
Feature C: Musculoskeletal Complications One of the following:

  • Musculoskeletal pain in ≥ 2 or more limbs, recurring daily for at least 3 months

  • Chronic, widespread pain for ≥ 3 months

  • Recurrent joint dislocations or frank joint instability, in the absence of trauma

Criterion 3:

All must be met

  • Absence of unusual skin fragility that should prompt consideration of other types of EDS

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

  • In patients with an acquired/autoimmune connective tissue disorder, must meet both Features A and B of Criterion 2. Feature C of Criterion 2 (chronic pain and/or instability) cannot be counted

  • Exclusion of alternative diagnoses that may also include joint hypermobility by means of hypotonia and/or connective tissue laxity