The three cases of hyper-IgE syndrome with coccidioidomycosis all had CNS involvement (87, 173, 174), while those with IL-12Rβ1 deficiency (139), IFN-γR1 deficiency (147), and GOF STAT1 defect (161) mainly had lymphadenopathy and osteomyelitis, so it appears that there is a predilection for Coccidioides to disseminate to the CNS in hyper-IgE syndrome. Here, IFNGR1 is linked to coccidioidomycosis.