MEFV and pyoderma gangrenosum: Demonstration of the interaction between the B-box/coiled-coil domains of pyrin with PSTPIP1, the protein that is mutated in PAPA syndrome (Pyogenic Arthritis, Pyoderma gangrenosum, and Acne), confirmed the important role played by pyrin in cytoskeletal signaling pathways (Shoham et al., 2003; Waite et al., 2009).