A mutation in exon 2 of the MEFV gene (c.726C > G; p.Ser242Arg) is associated with an autosomal dominant (AD) syndrome characterized by early onset of periodic fever, neutrophilic dermatosis, severe acne, pyoderma gangrenosum, sterile cutaneous abscesses and arthromyalgia, called pyrin-associated auto-inflammation with neutrophilic dermatosis (PAAND). Here, MEFV is linked to pyoderma gangrenosum.