Newborn with Severe Peeling of Skin: Bullous Congenital Ichthyosiform Erythroderma – To Treat or Not to Treat?

A 31-year-old lady with primary infertility conceived underwent in-vitro fertilization (IVF) for bilateral blocked fallopian tubes.She conceived in the first cycle itself with a single pregnancy.Her antenatal course was uneventful.She delivered a female baby of 2.5 kg with APGAR of 9/10.On delivery it was it was noted that baby had excessive peeling of skin all over her body.She was diagnosed with congenital ichthyosiform erythroderma.Unfortunately she succumbed.Her whole exome sequencing revealed pathogenic variant causative of KR10 gene (pathogenic) which is an autosomal dominant inheritance.The couple was advised with whole exome sequencing.