Hypomorphic hemizygous missense variants or short truncations of IKBKG in males that impair but do not abolish NF-kB signaling lead to hypohidrotic ectodermal dysplasia with immunodeficiency (EDA-ID, OMIM#300291) as well as immunodeficiency without EDA [4,5,6,7,8]. Here, IKBKG is linked to hypohidrotic ectodermal dysplasia.