Abstract
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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