The term PXE-like syndrome has been used to describe vascular, dermal, and ocular alterations characteristic of PXE that occur secondary to other diseases or due to genetic mutations different from those in ABCC6. These include hemoglobinopathies, such as beta-thalassemia or sickle cell disease (Aessopos et al., 2002), Paget’s disease of the bone (Gass and Clarkson, 1973), congenital dyserythropoietic anemia, ENPP1 mutations causing generalized arterial calcification of infancy (GACI) syndrome (Kalal et al., 2012), and mutations in GGCX (Li et al., 2009a). Here, GGCX is linked to body skin hyperlaxity due to vitamin K-dependent coagulation factor deficiency.