In physiological conditions, engagement of dectin-1 by β-glucans composing the fungi wall (1) triggers a complex signaling pathway mediating the oxidative burst (2) and the CARD9/BCL10/Malt1 complex formation (3), which in turn activates NF-κB, with secretion of proinflammatory cytokines (4), and Rac-1, inducing F-actin remodeling and mediating phagocytosis (5). Collectively, these processes lead to the pathogen clearance and consequently to the switching off of the inflammatory burst. In XIAP deficiency, dectin-1–induced innate responses is impaired. BCL10 fails to activate on one side, NF-κB, resulting in no secretion of NF-κB–mediated proinflammatory cytokines, and on the other side, Rac-1, resulting in no phagocytosis. Persistence of C albicans results in overactivation of macrophages, excess production of inflammatory cytokines, and hemophagocytic syndrome. Dashed lines represent the inactive molecules/processes of the pathway.