Netherton's Syndrome, an uncommon genodermatosis

Ikram-Ullah Khan, Rifat Chaudhary

Abstract


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.

 


Keywords


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

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References


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