Table 1.

Revised Diagnostic Criteria for Ehlers-Danlos Hypermobility Type, a.k.a. Joint Hypermobility Syndrome (JHS).

Revised Diagnostic Criteria for Ehlers–Danlos Hypermobility Type
Major Criteria:
  • A Beighton score of 4/9 or greater (either currently or historically)
  • Arthralgia for longer than 3 months in four or more joints
Minor Criteria:
  • A Beighton score of 1, 2, or 3/9 (0, 1, 2, or 3 if aged 50+)
  • Arthralgia (>3 months) in one to three joints or back pain (>3 months), spondylosis, spondylolysis/spondylolisthesis
  • Dislocation/subluxation in more than one joint, or in one joint on more than one occasion
  • Soft tissue rheumatism, >3 lesions (e.g. epicondylitis, tenosynovitis, bursitis)
  • Marfanoid habitus (tall, slim, span:height ratio >1.03, upper:lower segment ratio less than 0.89, arachnodactyly (positive Steinberg/wrist signs)
  • Abnormal skin: striae, hyperextensibility, thin skin, papyraceous scarring
  • Eye signs: drooping eyelids or myopia or antimongoloid slant
  • Varicose veins or hernia or uterine/rectal prolapse
JHS is diagnosed in the presence two major criteria, or one major and two minor criteria, or four minor criteria. Two minor criteria will suffice where there is an unequivocally affected first-degree relative.

Taken with permission from the Hypermobility Syndromes Association (HMSA) site (http://hypermobility.org/help-advice/hypermobility-syndromes/the-brighton-score/).50

RMMJ Rambam Maimonides Medical Journal Rambam Health Care Campus 2016 October; 7(4): e0034.
Special Issue on Rheumatology Guest Editor: Alexandra Balbir-Gurman, M.D.
ISSN: 2076-9172
Published online 2016 October 31. doi: 10.5041/RMMJ.10261.