Netherton's Syndrome, an uncommon genodermatosis

Ikram-Ullah Khan, Rifat Chaudhary


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.



Netherton syndrome, ichthyosis linearis circumflexa, trichorrhexis nodosa, trichorrhexis invaginata

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