Congenital Ichthyosis

See bonus NeoBriefs videos and downloadable teaching slides on this article’s homepage

Congenital ichthyosis (CI) encompasses a heterogenous group of skin disorders characterized by abnormal epidermal differentiation. Many present in the newborn period, and affected patients are at risk of serious complications. A compromised skin barrier places these infants at risk for transepidermal water loss, temperature dysregulation, compromised limb perfusion, and sepsis. It is important for neonatal clinicians to identify these disorders at birth and to create individualized management plans. This review seeks to summarize the signs of CI in the neonatal period and common management strategies.