The remaining 11 patients have familial cold autoinflammatory syndrome 4 (n = 1) [81]; deficiency of the interleukin 1 receptor antagonist (n = 1) [48]; pyogenic sterile arthritis, pyoderma gangrenosum, acne (PAPA) syndrome, hyperzincemia and hypercalprotectinemia (n = 1) [62]; mevalonate kinase deficiency (n = 1) [81]; SAMHD1 deficiency (n = 1) [95]; A20 deficiency (n = 1) [118]; Majeed syndrome (n = 1) [125]; STING-like disease (n = 1) [125]; CARD14 mediated psoriasis (n = 1) [125]; and unspecified autoinflammatory diseases (n = 2) [19, 40]. Here, CARD14 is linked to Familial cold urticaria.