Ikram Ullah Khan, Rifat Chaudhary.
Netherton`s syndrome, an uncommon Genodermatosis
J Pak Assoc Derma Jul - Sep 2006;16(3):180-2.
Pakistan Institute of Medical Sciences, Islamabad

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.

Category: Case Reports
Keywords: Netherton Syndrome. Ichthyosis Linearis Circumflexa. Trichorrhexis Nodosa. Trichorrhexis Invaginata.

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