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). The gene discussed is ABCC6; the disease is autosomal recessive inherited pseudoxanthoma elasticum.