A 2 year old born out of consanguineous marriage, had a history of the collodion membrane. He had large, thick, plate-like brown scaling generalized distribution; no erythroderma and ectropion. Which disease is it?

Ans. B. Lamellar ichthyosisLamellar ichthyosis (LI)* Collodion baby at birth as the baby is covered by a thickened collodion-like membrane which is then shed* Scaling occurs over the whole body, including creases and bends* The scale in LI is typically large, dark brown or grey and firmly adherent* Drooping of lower eyelids (ectropion), palmoplantar keratoderma and scarring alopecia may be seen in severe cases.* Limitation of joint movement, flexion contractures, and digital sclerodactyly may result. Nails may be small but hair shaft, mucous membranes and teeth are not affected.* May be associated with a mutation in transglutaminase 1 gene.