Netherton syndrome (NS) is a rare autosomal recessive disorder with an incidence of approximately one in 200,000 [1]. NS is characterized by the triad of congenital ichthyosiform erythroderma or ichthyosis linearis circumflexa, trichorrhexis invaginata (bamboo hair), and atopic diathesis (elevate serum IgE) [2,3]. The gene discussed is IGHE; the disease is Netherton syndrome.