Obstetrical aspects in congenital ichtyosis

Abstract

We present a case of congenital ichthyosis because obstetrical literature is scarce and most obstetricians could need a reminder and update. Congenital ichthyosis (CI) comprises a variety of skin disorders characterised by abnormal keratinization of the epidermis, which are mostly transmitted in an autosomal recessive manner. This condition is rare (seven per million people) with various clinical neonatal expressions and diversified prognosis, from self-healing to lethal. Even less severe phenotypes have significant associated morbidity and mortality. CI babies are often born prematurely and are at highest risk for complications during the postnatal period .CI, while fairly rare, is a condition well described in the literature, mainly from the neonatal point of view. We describe a case in which congenital ichthyosis was diagnosed after birth, and summarise the present literature with particular attention on obstetric implications as the prenatal diagnosis, genetic and ultrasound testing, perinatal complications and care for future pregnancies