Mutations in IKBKG can cause varying degrees of inactivation of the NF-κB signaling pathway, leading to different clinical manifestations; when the NF-κB pathway undergoes IKBKG amorphic mutations, it can cause incontinentia pigmenti (IP, OMIM 308300), which is an X-linked dominant genetic disease mainly occurring in female but lethal in male.3 Here, NFKB1 is linked to incontinentia pigmenti.