Inclusion criteria were an age older than 18 years and diagnosis of PXE according to modified Plomp criteria [16], namely, at least two among three of the following: (1) retinal anomalies such as peau d’orange and/or angioid streaks; (2) skin anomalies such as yellow papules or plaques in the lateral side of the neck or in the flexures of the axillae, elbows, or knees, or fragmentation and/or calcification of elastic fibers on a skin biopsy; and (3) biallelic pathogenic variants at the ABCC6 gene. The gene discussed is ABCC6; the disease is Pseudoxanthoma elasticum.