Keratosis Linearis with Ichthyosis Congenita and Sclerosing Keratoderma Syndrome – An Unusual Presentation

Keratosis linearis with ichthyosis congenita and sclerosing keratoderma syndrome is a rare autosomal recessive disorder which is characterised by palmoplantar keratoderma, linear hyperkeratotic plaques, ichthyosiform scaling, pseudoainhum and plaques distributed linearly in the flexures. A 7-year-boy presented with ichthyosiform scaling over body since birth, hyperkeratotic plaques over palms and soles. On cutaneous examination, diffuse ichthyosiform scales were present all over body with non-transgradient type of palmoplantar keratoderma with bilaterally symmetrical linear hyperkeratotic plaques over 5 th metatarsophalangeal joint. In our case, hyperkeratotic plaque was seen in extensor region over the feet. This case is being reported on account of its rarity and rare presentation.