Complications include bowel and arterial rupture and uterine rupture during pregnancy. Life expectancy is shortened with a majority of individuals living only into their 40's. The biochemical etiology is not known. These individuals have soft, velvety hyperextensible skin and hypermobile joints. Scarring is less severe.
Ocular fragility is common, as well as angioid streaking.
This is an autosomal recessive disorder. Unilateral and bilateral CDH is common. It is inherited as both an autosomal dominant and recessive disorder. There is a structural abnormality in the alpha 2 chains of type I collagen. Type VIII Ehlers-Danlos Syndrome, the periodontal form, shares with other variants, the cardinal features of cutaneous hyperextensibility and fragility, easy bruisability, dystrophic scarring, joint hypermobility and various manifestations of connective tissue weakness such as inguinal herniation.