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Lamellar ichthyosis (LI) is a keratinization disorder characterized by the presence of large scales all over the body without significant erythroderma. LI is a genetically heterogeneous disease. It is due to mutations in genes TGM1, ABCA12, ALOX12B, and NIPAL4. It is the most common variant of autosomal recessive congenital ichthyosis. Prevalence is estimated approximately at 1/100,000-1/1,000,000 individuals.