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Netherton Syndrome


Rare autosomal recessive disorder of cornification characterized by ichthyosis, hair shaft defects, and atopy. The infants show generalized erythema and scaling with failure to thrive. Later in infancy, polycyclic and serpiginous plaques develop which display double-edged scale along the margins. Atopic dermatitis with flexural lichenification and pruritus also may develop. Trichorrhexis invaginata (bamboo hair) is a characteristic feature, but it usually appears after infancy, as do pili torti and trichorrhexis nodosa.




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