Nonetheless, the pro-calcifying potential of vitamin deficiency and VKDP suppression should not be underestimated in PXE: pathogenic variants of the GGCX or VKORC1 genes may play a role in patients with severe or early onset disease; the usage of vitamin K antagonists should only be considered with extreme caution. Here, VKORC1 is linked to pseudoxanthoma elasticum (inherited or acquired).