Type	Beighton score	Musculoskeletal involvement*	Notes
Asymptomatic joint hypermobility
Asymptomatic generalized joint hypermobility	Positive	Absent	—
Asymptomatic peripheral joint hypermobility	Usually negative	Absent	Joint hypermobility typically limited to hands and/or feet
Asymptomatic localized joint hypermobility	Negative	Absent	Joint hypermobility limited to single joint or body parts
Hypermobility spectrum disorders
Generalized hypermobility spectrum disorders	Positive	Present	Does NOT meet criteria for hypermobile EDS based on limited findings in skin and musculoskeletal systems and lack of family history No genes identified Screening with echocardiography unnecessary
Peripheral hypermobility spectrum disorders	Usually negative	Present	Joint hypermobility typically limited to hands and/or feet
Localized hypermobility spectrum disorders	Negative	Present	Joint hypermobility limited to single joints or body parts
Historical hypermobility spectrum disorders	Negative	Present	Historical presence of joint hypermobility
EDS – Joint hypermobility with more pronounced skin and musculoskeletal findings and/or positive family history
1. Hypermobile EDS	Positive	Possible	Meet criteria based on supportive findings in skin and body systems and/or positive family history (see Figure 2) No genes identified AD inheritance pattern Obtain screening echocardiography
Type	Beighton score	Major features	Gene affected
EDS
2. Classical	Positive	Skin hyperextensibility Abnormal scarring	COL5A1, COL5A2 genes Rare COL1A1 gene AD inheritance
3. Classical-like	Positive	Skin hyperextensibility Easy bruising	TNXB gene AR inheritance
4. Cardiac-valvular	Positive or negative, general hypermobility or restricted to small joints	Cardiac valvular problems Skin involvement	COL1A2 gene AR inheritance
5. Vascular	Positive or negative	Family history of vascular EDS History of early arterial rupture or uterine rupture, sigmoid colon perforation, or atraumatic carotid-cavernous sinus fistula formation	COL3A1 gene Rare COL1A1 gene AD inheritance
6. Arthrochalasia	Positive	Congenital bilateral hip dislocation Skin hyperextensibility	COL1A1, COL1A2 genes AD inheritance
7. Dermatosparaxis	Positive or negative	Extreme skin fragility Characteristic craniofacial features	ADAMTS2 gene AR inheritance
8. Kyphoscoliotic	Positive with history of dislocation and subluxation	Congenital hypotonia Kyphoscoliosis	PLOD1, FKBP14 genes AR inheritance
9. Brittle cornea syndrome	Positive or negative	Thin cornea with or without rupture Keratoconus Keratoglobus Blue sclerae	ZNF469, PRDM5 genes AR inheritance
10. Spondylodysplastic	Positive or negative	Short stature Muscle hypotonia Bowing of limbs	B4GALT7, B3GALT6, SLC39A13 genes AR inheritance
11. Musculocontractural	Positive or negative	Congenital multiple contractures Characteristic craniofacial features Skin involvement	CHST14, DSE genes AR inheritance
12. Myopathic	Distal joints affected	Congenital muscle hypotonia and/or atrophy that improves with age Proximal muscle contractures	COL12A1 gene AD or AR inheritance
13. Periodontal	Positive or negative	Periodontitis Lack of attached gingiva Pretibial plaques Family history of periodontal EDS	C1R, C1S genes AD inheritance