In the 2017 EDS nosology, some patients with defects in two out of the five LK genes (i.e., B4GALT7 and B3GALT6) were grouped as spEDS together with those harboring SLC39A13 mutations, in consideration of the reliable clinical overlap [1], whereas the 22 Larsen of Reunion Island syndrome patients were not included. Here, SLC39A13 is linked to Ehlers-Danlos syndrome.