Variants that effectively reduce NF-κB activity, including hypomorphic variants in the IKK beta chain (encoded by IKBKB) or the IKK gamma chain (also known as NF-κB essential modifier, or NEMO, encoded by the IKBKG gene on the X chromosome), or activating variants in NFKBIA, cause a combined immunodeficiency with associated bone and skin findings. Here, NFKBIA is linked to Immunodeficiency.