Table 1: Classification of EDSs.

SubtypeInheritanceGene(s)

Major forms
 ClassicADCOL5A1, COL5A2
 Hypermobility/JHSAD?Mostly unknown
 VascularADCOL3A1
 KyphoscolioticARPLOD1
 ArthrochalasiaADCOL1A1, COL1A2
 DermatosparaxisARADAMTS2
Rare/emerging forms
 Tenascin X deficientAR, AD?TNXB
 Classic with vascular  ruptureADCOL1A1
 Cardiac-valvularARCOL1A2
 EDS/OI overlapADCOL1A1, COL1A2
 With periventricular  heterotopiaXLDFMNA
 MusculocontracturalARCHST14
 SpondylocheirodysplasticARSLC39A13
 ProgeroidARB4GALT7
 Kyphoscoliotic with  deafnessARFKBP14
 ParodontitisADUnknown
 Fibronectin deficientARUnknown

AD: autosomal dominant, AR: autosomal recessive, EDS/OI: Ehlers-Danlos syndrome/osteogenesis imperfect, JHS: joint hypermobility syndrome, XLD: X-linked dominant.