Analysis of data from the Prospective Lynch Syndrome Database (PLSD) has shown the cumulative risk at 75 years of age for PC is 6.2%, 0.5%, and 1.4%, and for BTC is 3.7%, 1.7%, and 0%, respectively, for carriers of MLH1, MSH2, and MSH6 germline variants (5). Here, MSH2 is linked to pachyonychia congenita.