This expands the cutaneous similarities between TNXB-clEDS and classical EDS; a fact that complicates the differential diagnosis of these disorders on clinical groups and reinforces the opportunity to consider TNXB molecular testing in all individuals with a clinical diagnosis of classical EDS resulted negative to COL5A1, COL5A2, and COL1A1 (recurrent variants) analysis. This evidence concerns the gene COL5A2 and Ehlers-Danlos syndrome due to tenascin-X deficiency.