BRCA carriers have an increased risk to develop PC (at 1.8- to 4.5-fold for BRCA1 carriers and at 2.5- to 8.6-fold for BRCA2 carriers in patients aged <65 years), in particular at early onset (Kote-Jarai et al., 2011; Leongamornlert et al., 2012). The gene discussed is BRCA2; the disease is pachyonychia congenita.