Hypohidrotic Ectodermal Dysplasia with Immunodeficiency, also known as anhidrotic ectodermal dysplasia with immunodeficiency, is related to ectodermal dysplasia and immune deficiency and incontinentia pigmenti. An important gene associated with Hypohidrotic Ectodermal Dysplasia with Immunodeficiency is IKBKG (Inhibitor Of Nuclear Factor Kappa B Kinase Regulatory Subunit Gamma), and among its related pathways/superpathways are TCR Signaling (Qiagen) and Beta-Adrenergic Signaling. Affiliated tissues include skin and eye, and related phenotypes are failure to thrive and hypohidrosis