<unk>  is inherited in an autosomal dominant fashion, which means that if one parent is affected, there is a 100% chance that a child will inherit the disease from a homozygous affected parent and a 50% chance that a child will inherit the disease from an affected heterozygous parent. 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.
PAPA syndrome
PEHO syndrome
Saal Greenstein syndrome
Crandall syndrome
PHACE syndrome
Arts syndrome
Mietens syndrome
ANOTHER syndrome