“…This leads to patients presenting with disturbed IL-17 immunity, with significant Th17 cytopenia [36,48,50] with an associated impairment in IL-17A and IL-22 production [52], but also defects in B cell differentiation [53]. There is proinflammatory skewing, with reduced tolerogenic function of dendritic cells (DCs) [54], monocytes polarized toward a proinflammatory state with enhanced responsiveness to Tolllike receptor (TLR) 7/8 stimulation [55] and neutrophils with inflammatory markers [56]. Patients with these mutations represent around 50% of all cases of chronic mucocutaneous candidiasis (CMC), which is characterized by recurrent/persistent mucocutaneous infection by Candida fungi [36,57], but also exhibit increased susceptibility to bacterial, viral and other fungal infections of the lower respiratory tract, autoimmune manifestations, such as enterocolitis, systemic lupus erythematosus and relevant thyroid diseases [46].…”