Although the absence of infections made classic EDA with immune deficiency (EDA-ID) unlikely, the combination of immune dysregulation coupled with abnormal tooth development suggested a possible underlying functional defect in NEMO, IκBα, or a functionally related gene, which could disrupt ectodysplasin signaling and produce the observed dental phenotype. This evidence concerns the gene IKBKG and hypohidrotic ectodermal dysplasia.