Outcomes of Systemic Treatment in Children and Adults With Netherton Syndrome: A Systematic Review.

ABSTRACT:

Background

Comèl-Netherton syndrome (NS) is a rare disease caused by pathogenic variants in the SPINK5 gene, leading to severe skin barrier impairment and proinflammatory upregulation. Given the severity of the disease, treatment of NS is challenging. Current treatment regimens are mainly topical and supportive. Although novel systemic treatment options for NS have been suggested in recent literature, little is known about their outcomes.

Objective

to provide an overview of systemic treatment options and their outcomes in adults and children with NS.

Methods

Embase, MEDLINE, Web of Science, Cochrane Central Register of Controlled Trials, and Google Scholar were searched up to July 22, 2021. Empirical studies published in English language mentioning systemic tre

SUBMITTER: Nouwen AEM 

PROVIDER: S-EPMC9022473 | biostudies-literature | 2022

REPOSITORIES: biostudies-literature