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Year : 2016  |  Volume : 5  |  Issue : 3  |  Page : 185-187

Netherton syndrome presenting as recurrent erythroderma: A rare case report

Department of Dermatology, Venereology and Leprosy, Vydehi Institute of Medical Sciences, Bengaluru, Karnataka, India

Correspondence Address:
Dr. K G Manjunath
Vydehi Hospital, # 82, EPIP Area, Whitefield, Bengaluru - 560 066, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2278-344X.187834

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Netherton syndrome (NS) is a rare autosomal recessive ichthyosiform disease. The incidence is 1 in 200,000. It is caused by mutation in the serine protease inhibitor Kazal-type 5 gene which encodes the lymphoepithelial Kazal type-related inhibitor. It presents with ichthyosis linearis circumflexa, trichorrhexis invaginata, and atopy. A 20-year-old male presented with erythroderma and further examination revealed atopic features. Dermoscopy was a convenient tool in identifying trichorrhexis nodosa. We are reporting a case of NS presenting as recurrent erythroderma.

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