| PMC full text: |
|
Fig 1.
Brighton criteria for the diagnosis of BJHS.
| Major Criteria |
| • Beighton score of ≥ 4 (Fig. 2) |
| • Arthralgia for longer than 3 months in 4 or more joints |
| Minor Criteria |
| • Beighton score of 1, 2, or 3 (Fig. 2) |
| • Arthralgia (> 3-month duration) in 1-3 joints or back pain (> 3-month duration) or spondylosis, spndylolysis/spondylolisthesis. |
| • Dislocation or subluxation in more than one joint, or in one joint on more than one occasion. |
| • Three or more soft tissue lesions (eg, epicondylitis, tenosynovitis, bursitis) |
| • Marfanoid habitus (tall, slim, span greater than height [> 1.03 ratio], upper segment less than lower segment (< 0.89 ratio), arachnodactyly) |
| • Skin striae, hyperextensibility, thin skin, or abnormal scarring |
| • Ocular signs: drooping eyelids, myopia, antimongoloid slant |
| • Varicose veins, hernia, or uterine or rectal prolapse, mitral valve prolapse |
| Requirement for diagnosis BJHS is diagnosed in the presence of 2 major criteria, one major criterion plus 2 minor criteria, or 4 minor criteria. Two minor criteria will suffice where there is an unequivocally affected first-degree relative in family history. The syndrome is excluded by the presence of Marfan or Ehlers-Danlos syndrome (other than the hypermobility type of Ehlers-Danlos syndrome, formerly EDS III). |