Table 1.

Classification of Ehlers–Danlos syndrome (Villefranche Nosology).

Ehlers–Danlos syndrome – Type	Inheritance	Clinical features	Reported pregnancy-related problems
Classical (Type 1 and 2)	AD	Skin hyperextensibility, easy bruising, tissue friability, joint hypermobility	Preterm labour, PPROM, perineal trauma, PPH
Hypermobility (Type 3)	AD	Generalised joint hypermobility, recurrent joint dislocation/pain, mild cutaneous manifestations	PoTS, preterm labour, PPROM, perineal trauma, PPH
Vascular (Type 4)	AD	Facial dysmorphism, thin translucent skin, poor wound healing, excessive bruising/haematomas, vascular rupture and intestinal perforation	Mortality around 12%. Risk of arterial or uterine rupture, PPH requiring hysterectomy
Kyphoscoliotic (Type 6)	AR	Soft velvety skin, poor scarring, joint-laxity, dislocations and kyphosis, ocular fragility, vascular rupture, mitral valve prolapse	Miscarriage, preterm labour, PROM, arterial rupture
Arthrochalasia (Type 7A-B)	AD	Severe generalised joint hypermobility and dislocations, floppy infants, scoliosis, mild skin features
Dermatosparaxis (Type 7C)	AD	Severe skin manifestations – fragility, bruising, poor scarring, visceral rupture	PPROM and preterm labour, lacerations and fractures at birth

AD: autosomal dominant; AR: autosomal recessive; PPROM: preterm prelabour rupture of membranes; PPH: postpartum haemorrhage; PoTS: postural orthostatic tachycardia syndrome.

Inheritance pattern, clinical features and pregnancy-related problems (adapted from (1,2)).