The mutations of NEMO are associated to two clinically different inherited genetic diseases, Anhidrotic Ectodermal Dysplasia, with ImmunoDeficiency (EDA-ID, OMIM#300291) and Incontinentia Pigmenti (IP, OMIM#308300), depending on the genetic state and on their effects on the impairment of NF-κB activation. Here, IKBKG is linked to incontinentia pigmenti.