The updated classification of EDS linked collagen type I pathogenic variants include: arthrochalasia EDS–aEDS (associated to COL1A1 and COL1A2 mutations); cardiac-valvular–cvEDS (related to biallelic pathogenic COL1A2 variants); vascular EDS–vEDS (rarely associated to specific COL1A1 arginine to cysteine substitutions); and classic EDS–cEDS (rarely related to COL1A1) (Malfait et al., 2017). This evidence concerns the gene COL1A1 and Ehlers-Danlos syndrome.