From:  The significance of hypermobility syndromes for rheumatology

 Phenotypes belonging to the hypermobility spectrum.

PhenotypeSymptomsBeighton scoreMusculoskeletal involvement
g-HMAsymptomaticPositiveNo
p-HMAsymptomaticNegativeNo
l-HMAsymptomaticUsually negativeNo
g-HSDYesPositiveYes
p-HSDPossibleMostly negativeYes
l-HSDPossibleNegativeYes
h-HSD*PossibleNegativeYes
hEDS**YesPositivePossible

*History of joint hypermobility (e.g., positive 5-item questionnaire); ** plus a positive family history (first-degree relatives) and/or specific systemic manifestations (see new criteria). All these phenotypes require careful exclusion of other acquired and hereditary disorders, some of which overlap. (a) Peripheral joint hypermobility is typically limited to the hands and/or feet; the Beighton score is usually negative. (b) Localised joint hypermobility is restricted to individual joints or body parts; the Beighton score is negative. (c) See the new criteria for the operational definition of ‘generalised joint hypermobility’ as a mandatory feature/criterion of hypermobile Ehlers-Danlos syndrome. g-HM: generalised hypermobility disorder; g-HSD: generalised hypermobility spectrum disorder; hEDS: hypermobile Ehlers-Danlos syndrome; l-HSD: localised hypermobility spectrum disorder; p-HM: peripheral joint hypermobility; p-HSD: peripheral hypermobility spectrum disorder.