The observation of abnormally mineralized skin areas in a woman with a p.R1141X mutation in ABCC6 and a p.V255M mutation in GGCX (coding for gamma-glutamyl carboxylase) [64] has prompted the consideration of a forme fruste of PXE (OMIM #177850). Here, ABCC6 is linked to pseudoxanthoma elasticum (inherited or acquired).