Netherton's syndrome

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A disorder of hair (‘bamboo hair’, trichorrhexis nodosa) and skin (ichthyosiform erythroderma) caused by mutation in the gene for the serine protease inhibitor, LEKTI (lymphoepithelial Kazal-type-related inhibitor), expressed in epithelial and mucosal surfaces and in the thymus.

Subjects: Medicine and Health.

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