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DERMACON 2016: Rare Genodermatoses - Netherton Syndrome
Netherton syndrome is a rare autosomal recessive hereditary ichthyosiform disease. In this disease, mutation is seen in the serine protease inhibitor, Kazal type 5 gene (SPINKS), located on the long arm of Chromosome 5. The classical triad includes ichthyosiform dermatosis, hair shaft abnormalities and atopic diathesis.
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