Netherton syndrome: Temporary response to dupilumab

被引：30

作者：

Aktas, Meryem ^{[1
]}

Salman, Andac ^{[1
]}

Apti Sengun, Ozlem ^{[1
]}

Comert Ozer, Elif ^{[1
]}

Hosgoren Tekin, Selcen ^{[1
]}

Akin Cakici, Ozlem ^{[1
]}

Demir, Gizem ^{[1
]}

Ergun, Tulin ^{[1
]}

机构：

[1] Marmara Univ, Sch Med, Dept Dermatol, Istanbul, Turkey

来源：

PEDIATRIC DERMATOLOGY | 2020年 / 37卷 / 06期

关键词：

atopic eczema; genetic diseases; mechanisms; Ichthyosis;

D O I：

10.1111/pde.14362

中图分类号：

R75 [皮肤病学与性病学];

学科分类号：

100206 ;

摘要：

Netherton syndrome (NS) is an orphan disease characterized by congenital ichthyosis, hair abnormalities, and atopy, with limited treatment options. We achieved temporary improvement only during the initial 6 weeks of treatment with dupilumab, which differs from the sustained improvement observed in 2 other recently published cases. Although the clinical presentation of atopy and increased pre-allergic cytokines in NS patients suggest that dupilumab may be beneficial, larger studies are required.

引用

页码：1210 / 1211

页数：2

共 5 条

[1] Successful treatment with dupilumab of an adult with Netherton syndrome
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Steuer, Alexa B.
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[J]. JAMA DERMATOLOGY, 2020, 156 (03) : 350 - 351

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