They include anhidrotic ectodermal dysplasia with immunodeficiency [8] [of which there are two forms, X-linked and autosomal dominant, caused by defects in NF-kappa-B essential modulator (NEMO) or gain-of-function in alpha inhibitor ofNF-kappa-B (IKBA)], and the IL-1 receptor-associated kinase type 4 (IRAK-4) deficiency [7–12] and myeloid differentiation primary response 88 (MyD88) deficiency [11–12], both autosomal recessive. The gene discussed is IKBKG; the disease is Immunodeficiency.