Allergy Service, Hospital Universitario de Salamanca, Spain.
Departamento de Ciencias Biomédicas y del Diagnóstico, Facultad de Medicina, Universidad de Salamanca, Spain.
Int J Immunopathol Pharmacol. 2023 Jan-Dec;37:3946320231172881. doi: 10.1177/03946320231172881.
Different monoclonal antibodies have been used for the treatment of Netherton's syndrome (NS); secukinumab (anti-IL17A), infliximab (anti-TNF-α), ustekinumab (anti p40 subunit of IL-12 and IL-23), omalizumab (anti-IgE), and dupilumab (anti-IL4 and IL13). We report two sisters with severe NS who were treated with omalizumab in one and with secukinumab in the other. In view of the therapeutic failure, treatment with dupilumab was started in both sisters. The data were analyzed 16 weeks after starting treatment with dupilumab. Treatment response was assessed using the Severity Scoring Atopic Dermatitis (SCORAD); Eczema Area and Severity Index (EASI); Pruritus Numeric Rating Scale (NSR); Netherton Area Severity Assessment (NASA) and Dermatology Life Quality Index Ichthyosis. All scores were reduced after 16 weeks of treatment with dupilumab in both patients. She maintains improvement after 18 months and 12 months of treatment, respectively. No severe adverse events were reported. Treatment with dupilumab in two sisters with NS and atopic diseases produced a marked cutaneous improvement after a failed attempt with omalizumab and secukinumab. Further studies are needed to determine which biologic therapy is the most effective in NS.
不同的单克隆抗体已被用于治疗 Netherton 综合征（NS）；司库奇尤单抗（抗 IL-17A）、英夫利昔单抗（抗 TNF-α）、乌司奴单抗（抗 p40 亚单位的 IL-12 和 IL-23）、奥马珠单抗（抗 IgE）和度普利尤单抗（抗 IL-4 和 IL-13）。我们报告了 2 例严重 NS 姐妹，其中 1 例接受奥马珠单抗治疗，另 1 例接受司库奇尤单抗治疗。鉴于治疗失败，开始在两姐妹中使用度普利尤单抗治疗。在开始使用度普利尤单抗治疗 16 周后分析了数据。使用特应性皮炎严重程度评分（SCORAD）、湿疹面积和严重程度指数（EASI）、瘙痒数字评分量表（NSR）、Netherton 区域严重程度评估（NASA）和皮肤病生活质量指数鱼鳞病来评估治疗反应。两名患者在接受度普利尤单抗治疗 16 周后，所有评分均降低。她们分别在治疗 18 个月和 12 个月后仍保持改善。未报告严重不良事件。在奥马珠单抗和司库奇尤单抗治疗失败后，两例 NS 和特应性疾病的姐妹接受度普利尤单抗治疗后，皮肤明显改善。需要进一步研究以确定哪种生物疗法对 NS 最有效。
