Several defects of the skin barrier frequently cause secondary infection of the affected epithelia and can also present with additional features resembling known IEIs105, such as Netherton syndrome (caused by autosomal recessive [AR] defects inSPINK5), hyper IgE syndrome (caused by defects inSTAT3,DOCK8,IL6ST,ZNF341, or phosphoglucomutase 3 [PGM3]), or immunodysregulation polyendocrinopathy enteropathy X-linked (IPEX) syndrome (caused by X-linkedFOXP3 defects). The gene discussed is PGM3; the disease is infection.