Moreover, as in the case of the Comèl–Netherton syndrome [AR serine peptidase inhibitor Kazal type 5 (SPINK5) defect], HIES may be related to a skin barrier disruption showing congenital ichthyosis, bamboo hair, atopic diathesis, increased bacterial infections with impaired responses to polysaccharide vaccination, enteropathy, and failure to thrive [25]. Here, SPINK5 is linked to Netherton syndrome.