Table 1: Villefranche classification of Ehlers-Danlos syndrome (1998).

TypeInheritanceGene(s)Phenotype

Classic EDS (Types I/II)ADCOL5A1,
COL5A2
Hyperelastic, soft skin, atrophic scars, easy bruising, joint hyperlaxity
Hypermobility EDS (Type III)ADUnknownGross joint hyperlaxity, mild atrophic scarring and easy bruising
Vascular EDS (Type IV)ADCOL3A1Typical facial gestalt, skin fragility, extreme vascular fragility, rupture of uterus and colon
Kyphoscoliosis EDS (Type VI)ARPLODMarfanoid habitus, hypotonia, kyphoscoliosis, ocular complications + features of Type I EDS
Arthrochalasis EDS
(Types VIIA and B)
ADCOl1A1,
COL1A2
Severe joint hyperlaxity, congenital bilateral hip dyslocation, easy bruising, scoliosis, hypotonia
Dermatosparaxis EDS
(Type VII C)
ARProcollagen,
N-peptidase
Severe skin fragility, sagging redundant skin, excessive bruising

AD: autosomal dominant; AR: autosomal recessive.