Genetic factors (e.g., CARD15/ NOD2 mutation) may play a role by interacting with mycobacterial/bacterial/viral components, promoting the activation of NF-κB and tumor necrosis factor receptor-associated factor 3 (TRAF3) signaling pathways and triggering granulomatous autoinflammation in BS/EOS patients [22,23,24]. This evidence concerns the gene NFKB1 and Blau syndrome.