Congenital ichthyosiform erythroderma in pregnancy: a case report of a retinoic acid embryopathy

Congenital ichthyosiform erythroderma (EHK) is a major cutaneous disease, autosomal dominant, usually present at birth. There is no curative treatment; in the most severe cases, the control of symptoms relies on oral retinoids. We present a case of a pregnant woman with EHK taking retinoids until the 33rd week of pregnancy. The woman delivered a newborn with multiple minor malformations, without cutaneous lesions, suggestive of retinoic acid embryopathy. This case highlights the importance of recognizing the teratogenicity of drugs in women of reproductive age. We describe a complex multidisciplinary approach and the obstetrical challenges in such a rare disease.