Abstract: A 5-year-old boy presented with diffuse dry, desquamation and pruritus after birth. The patient was born with dry, flaky skin on the forehead, hands and lower legs, gradually spreading to the femur and abdomen. After the age of 2, the skin of the whole body began to be diffusely erubescent, exacerbated by scales and intense itching. The trunk and limbs were covered with large amounts of scales, with local infiltration and hypertrophy, and the skin on the extensor sides of the joints was markedly mossy. Dermoscopic examination showed bamboo hair. Blood eosinophils was 1.05×109/L. IgE＞1130.00 IU/mL. The diagnosis of Netherton syndrome was made. At present, there are 15 literatures about the clinical effect of dupliuzumab in the treatment of NS. This patient was treated with dupilumab,  itching improved significantly and the generalized scales was briefly relieved after 4 months. In this paper, the previous literature and the treatment of this patient are reviewed.

Key words: Netherton syndrome, bamboo hair, dupilumab