Department of Dermatology and Venereology, İstanbul Faculty of Medicine, İstanbul University, İstanbul, Turkey.
Department of Dermatology and Venereology, Faculty of Medicine, Çanakkale Onsekiz Mart University, Çanakkale, Turkey.
Australas J Dermatol. 2023 May;64(2):272-277. doi: 10.1111/ajd.13986. Epub 2023 Feb 6.
We herein present a unique patient of Netherton syndrome (NS) with ichthyosis linearis circumflexa (ILC) lesions associated with severe atopic manifestations since infancy, showing different responses of atopic and ILC lesions to a 2-year dupilumab therapy. The atopic eczematous lesions and pruritus healed remarkably, dramatically improving the patient's quality of life, whilst the scalp hair showed a clinical and light microscopic improvement. The additional recovery in axillary/pubic/extremity hair growth, sweating and nail growth in the presented case was not previously reported in NS patients treated with dupilumab. However, dupilumab had no therapeutic effect on ILC lesions which were not pruritic and showed a treatment-independent wax and waned course.
我们在此介绍一例 Netherton 综合征（NS）患者，其具有回旋状线性鱼鳞病（ILC）病变，自婴儿期起即伴有严重特应性皮炎表现，在接受为期 2 年的度普利尤单抗治疗后，特应性皮炎和 ILC 病变出现不同的反应。特应性皮炎样皮损和瘙痒显著改善，患者的生活质量明显提高，而头皮毛发表现出临床和光镜下的改善。在本病例中，腋部/阴部/四肢毛发的生长、出汗和指甲生长出现了额外的恢复，这在接受度普利尤单抗治疗的 NS 患者中尚无报道。然而，度普利尤单抗对无瘙痒且呈非治疗依赖性时轻时重病程的 ILC 病变无治疗作用。
