Naegeli-Franceschetti-Jadassohn syndrome (NFJS), also recognized as Naegeli Syndrome, is a distinctive autosomal dominant disorder that arises from mutations in Keratin 14 (KRT14) gene, disrupting the normal development and differentiation of ectodermal tissues. The gene discussed is KRT14; the disease is Naegeli-Franceschetti-Jadassohn syndrome.