Comparable TEM findings in individuals with dEDS and aEDS due to specific COL1A1 and COL1A2 variants are understandable as dEDS is due to deficient activity of ADAMTS-2, an endopeptidase that excises the N-propeptide of procollagen chains [19, 20]. Here, COL1A2 is linked to Ehlers-Danlos syndrome, dermatosparaxis type.