Ichthyosis prematurity syndrome (IPS) is a rare form of autosomal recessive congenital ichthyosis caused by mutations in SLC27A4 (OMIM: 604194) encoding fatty acid transport protein 4 (FATP4) that affect keratinocyte differentiation and skin barrier formation. It is characterized by prematurity, thick caseous desquamating epidermis, and perinatal respiratory asphyxia. Despite its well-described clinical features and major perinatal mortality risk, IPS is still rarely recognized, and only a few cases have been reported.
Lwin SM, Hsu C, McMillan JR, Mellerio JE, McGrath JA. Ichthyosis Prematurity SyndromeFrom Fetus to Adulthood. JAMA Dermatol. 2016;152(9):1055-1058. doi:10.1001/jamadermatol.2016.1187