Hereditary pancreatitis associated with mutations in the cationic trypsinogen gene, PRSS1 (143), SPINK1, PRSS2, and CTRC, also lead to an increased risk ranging between 26- and 70-fold compared to the general population, with a cumulative risk of 40–55% by age 70 for developing PC (60, 91–93). Here, PRSS1 is linked to pachyonychia congenita.