度普利尤单抗治疗Netherton综合征一例并文献复习

A case of Netherton syndrome treated with dupilumab and literature review

患者,男,5岁。全身皮肤干燥、脱屑,痒4年。患者出生20天后自前额、手、小腿开始出现干燥、脱屑,逐渐蔓延至股部、腹部。2岁后全身皮肤开始弥漫潮红、脱屑加重并伴剧烈瘙痒。躯干、四肢覆大量鳞屑,局部浸润、肥厚,关节伸侧皮肤显著苔藓化。皮肤镜下眉毛呈竹节状改变。血常规示嗜酸性粒细胞1.05×109/L。免疫球蛋白E>1130.00 IU/mL。诊断:Netherton综合征。目前有15篇文献报道应用度普利尤单抗治疗NS的临床效果,本例患者应用度普利尤单抗治疗4个月,患者瘙痒情况明显改善,全身脱屑短暂缓解,本文对既往文献及本例患者治疗进行了回顾性分析。

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.