A different group of genes encoding the transforming growth factor beta (TGF-β) signaling pathway (TGF-β receptors 1 and 2 (TGFBR1, TGFBR2), TGFB2, TGFB3, similar to Mothers Against Decapentaplegic 2 and 3 (SMAD2, SMAD3) and Sloan–Kettering Institute Proto-Oncogene (SKI)) give rise to TGF-β-related vascular disorders, which are linked to LDS (especially TGFBR1 or TGFBR2 mutations), vEDS (mutations in collagen type III alpha 1 chain gene (COL3A1)) and predispose patients to aggressive and widespread vascular conditions [5]. This evidence concerns the gene TGFBR1 and Ehlers-Danlos syndrome, vascular type.