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. Author manuscript; available in PMC: 2022 Nov 21.
Published in final edited form as: Vasc Med. 2022 Jan 10;27(3):283–289. doi: 10.1177/1358863X211067566

Table 1.

Definition of hypermobile Ehlers–Danlos syndrome and hypermobility spectrum disorder diagnoses.

Diagnosis Main diagnostic features
Hypermobile Ehlers-Danlos syndrome (hEDS) Generalized joint hypermobility (positive Beighton score), positive family history, and/or specific systemic manifestations
Generalized hypermobility spectrum disorder (G-HSD) Generalized joint hypermobility (positive Beighton score) with musculoskeletal involvement
Localized hypermobility spectrum disorder (L-HSD) Localized joint hypermobility (negative Beighton score) with musculoskeletal involvement
Historical hypermobility spectrum disorder (H-HSD) Historical presence of joint hypermobility (e.g., positive 5-point questionnaire) but negative Beighton score, musculoskeletal involvement
Asymptomatic generalized joint hypermobility (A-GJH) Generalized joint hypermobility, no musculoskeletal involvement

Diagnostic criteria are based on Malfait et al., 20172 and Castori et al. 2017.15 All diagnoses are based on exclusion of other acquired and hereditary connective tissue disorders or genetic conditions that may have overlapping features, on either clinical or molecular diagnostic grounds.