Ehlers-Danlos Syndrome, Hypermobility Type: An Underdiagnosed Hereditary Connective Tissue Disorder with Mucocutaneous, Articular, and Systemic Manifestations

Table 3

The Brighton criteria for JHS.

The Brighton criteria
Major criteria
Beighton score ≥ 4/9
Arthralgia for >3 months in >4 joints
Minor criteria
Beighton score of 1–3
Arthralgia in 1–3 joints
History of joint dislocations
Soft-tissue lesions > 3
Marfan-like habitus
Skin striae, hyperextensibility, or scarring
Eye signs, lid laxity
History of varicose veins, hernia, visceral prolapse

Adapted from [9].
Note 1: criteria major 1 and minor 1 are mutually exclusive as are major 2 and minor 2.
Note 2: for the diagnosis of the joint hypermobility syndrome: both major, or 1 major and 2 minor, or 4 minor criteria, or 2 minor criteria and one or more first-degree affected relative(s).
Note 3: diagnosis of joint hypermobility syndrome needs previous (clinical/molecular) exclusion of other overlapping heritable connective tissue disorders, such as Marfan syndrome and other Ehlers-Danlos syndrome subtypes.