The most frequently mutated gene in vEDS is COL3A1, which encodes for collagen type III, followed by COL1A1, which is less common and encodes for collagen type I. Vascular EDS is characterized by arterial fragility with aneurysm development, dissection, and rupture, and in general by organ fragility, extensive bruising, and pneumothorax. Here, COL3A1 is linked to Ehlers-Danlos syndrome, vascular type.