With the growing interest in identifying the causal factors responsible for the development and progression of PC [13,16,21,22], phosphatidylinositol-3,4,5-trisphosphate-dependent rac exchange factor 2 (PREX2) and lysine demethylase 6A (KDM6A) have recently been identified as two new drivers of pancreatic tumorigenesis [23]. Here, PREX2 is linked to pachyonychia congenita.