Recently, Iwanaga et al. [14] reported an ABCC6 mutation analysis study in 76 Japanese patients with PXE revealing that 56 (80%) of 76 patients had eye complications that were likely attributed to AS, while our study showed that PXE was seen in 15 of 17 AS patients (88.2%) who underwent dermatological examination. Here, ABCC6 is linked to Pseudoxanthoma elasticum.