The dermatosparaxis (ADAMTS2) [61], cardiac-valvular (COL1A2) [62–64], kyphoscoliotic (PLOD1, FKBP14) [65, 66], and arthrochalasia (COL1A1, COL1A2) [67, 68] EDS subtypes, also sharing with cEDS several cutaneous and articular issues, are mostly distinguishable for the presence of specific hallmarks [1, 9, 16]. This evidence concerns the gene ADAMTS2 and Ehlers-Danlos syndrome.