In these monogenic autoinflammatory syndromes, enhanced activation of the inflammasome pathway due to genetic mutations (i.e., nucleotide-binding oligomerization domain, Leucine-rich Repeat and Pyrin domain 1/NLRP1, NLRP3, NLR family CARD domain-containing protein 4/NLRC4 or Pyrin), leads to common clinical phenotypes involving fever, skin rash, or dermatologic lesions and systemic symptoms (such as arthralgia or arthritis for example), associated with elevated inflammatory markers in the blood of affected patients [11]. This evidence concerns the gene NLRC4 and autoinflammatory syndrome.