 Recently the responsible gene has been identified on Chromosome 15.Two mutations have been found in a protein called CD2 binding protein 1 (CD2BP1).This protein is part of an inflammatory pathway associated with other autoinflammatory diseases such as familial Mediterranean fever, Hyperimmunoglobulinemia D with recurrent fever, Muckle–Wells syndrome, neonatal onset multisystem inflammatory disease, and familial cold urticaria.