Netherton s Syndrome, an uncommon genodermatosis

Netherton s syndrome (NS) is the most common of the multisystem ichthyosiform syndromes, and comprises an icthyosiform dermatosis with a variable erythroderma, hair shaft defects and atopic features. Mutation in type 5 (SPINKS) gene has been identified in several NS patients. We report a young male who had perioral erythema, ichthyosiform lesion and hair abnormalities. He was diagnosed on clinical as well as histological ground