What is the subtype of Ehlers-Danlos syndrome associated with the highest risk?

The vascular type of Ehlers-Danlos syndrome (EDS) is associated with the highest risk primarily due to its association with serious cardiovascular complications. This subtype is characterized by a deficiency in type III collagen, which is critical for the structural integrity of blood vessels and organs. As a result, individuals with this type face an increased risk of vascular rupture, especially of the aorta, and other internal organs, which can lead to life-threatening conditions.

Patients with the vascular type may also present with other serious complications such as spontaneous organ rupture and severe bruising due to the fragile vasculature. In contrast, the other subtypes of EDS, while they may present with their own issues such as joint hypermobility and skin elasticity, do not carry the same level of acute risk for vascular or organ rupture as seen in the vascular type. Thus, understanding the unique risks associated with different types of EDS is crucial for appropriate management and counseling of affected patients.