A better interpretation of FMF mutations in various clinical sceneries has led to coin the umbrella-term “pyrin-associated autoinflammatory diseases”, including all non-FMF diseases caused by MEFV-related pyrin defects, such as periodic undefined fevers, periodic fever/aphthosis/pharyngitis/adenitis syndrome-like pictures and neutrophilic dermatosis [42]. Here, MEFV is linked to familial Mediterranean fever.