Type	IP*	Major Criteria
Classical EDS - cEDS	AD	Skin hyperextensibility and atrophic scarring; and generalized joint hypermobility (GJH).
Classical-like EDS - clEDS	AR	Skin hyperextensibility with velvety skin texture and absence of atrophic scarring; GJH with or without recurrent dislocations (most often shoulder and ankle); and easily bruised skin or spontaneous ecchymoses (discolorations of the skin resulting from bleeding underneath).
Cardiac Valvular EDS - cvEDS	AR	Severe progressive cardiac-valvular problems (aortic valve, mitral valve); skin involvement: skin hyperextensibility, atrophic scars, thin skin, easy bruising; and joint hypermobility (generalized or restricted to small joints).
Vascular EDS - vEDS	AD	Family history of vEDS with documented causative variant in COL3A1; arterial rupture at a young age; spontaneous sigmoid colon perforation in the absence of known diverticular disease or other bowel pathology; uterine rupture during the third trimester in the absence of previous C-section and/or severe peripartum perineum tears; and carotid-cavernous sinus fistula formation in the absence of trauma.
Hypermobile EDS - hEDS	AD	The diagnosis of hypermobile EDS (hEDS) remains clinical; there is no molecular, genetic cause yet identified, so there is no test available for almost all with hEDS. Feature A—systemic manifestations of a more generalized connective tissue disorder (a total of five out of twelve must be present) Feature B—positive family history, with one or more first degree relatives independently meeting the current diagnostic criteria for hEDS Feature C—musculoskeletal complications
Arthrochalasia EDS - aEDS	AD	Congenital bilateral hip dislocation; severe GJH, with multiple dislocations/subluxations (medical); and skin hyperextensibility.
Dermatosparaxis EDS - dEDS	AR	There are nine major criteria and eleven minor criteria. Minimal criteria suggestive of dEDS include the two major criteria of extreme skin fragility and characteristic craniofacial features, plus either: one other major criterion, or three minor criteria.
Kyphoscoliotic EDS - kEDS	AR	Congenital muscle hypotonia; congenital or early onset kyphoscoliosis (progressive or non-progressive); and GJH with dislocations/subluxations (medical) (shoulders, hips and knees in particular).
Brittle Cornea Syndrome - BCS	AR	Thin cornea, with or without rupture (central corneal thickness often <400 µm); early onset progressive keratoconus; early onset progressive keratoglobus; and blue sclerae.
Spondylodysplastic EDS - spEDS	AR	Short stature (progressive in childhood); muscle hypotonia (ranging from severe congenital, to mild later-onset); and bowing of limbs.
Musculocontractural EDS - mcEDS	AR	Congenital multiple contractures, characteristically adduction-flexion contractures and/or talipes equinovarus (clubfoot); characteristic craniofacial features, which are evident at birth or in early infancy; and characteristic cutaneous features including skin hyperextensibility, easy bruisability, skin fragility with atrophic scars, increased palmar wrinkling.
Myopathic EDS - mEDS	AD or AR	Congenital muscle hypotonia, and/or muscle atrophy, that improves with age; proximal joint contractures (knee, hip and elbow); and hypermobility of distal joints.
Periodontal EDS - pEDS	AD	Severe and intractable periodontitis of early onset (childhood or adolescence); lack of attached gingiva; pretibial plaques; and family history of a first-degree relative who meets clinical criteria.