Netherton syndrome —A therapeutic challenge in childhood

We describe an 8-year-old boy with genetically proven NS treated with intravenous immunoglobulin for recurrent skin and systemic infections from infancy, growth retardation, and associated erythroderma. Under this therapy, his skin status, infectious exacerbations, and quality of life all improved. Knowledge of the cytokine-mediated pathogenesis of NS and the development of new biologic drugs open new possibilities for NS patients. However, the different therapeutic options have been applied in a limited number of cases, and variable responses have been shown. Randomized controlled trials with a sufficient number of patients stratified and treated according to their specific immune profile and clinical phenotype are needed to evaluate the safety and efficacy of treatment options for patients with NS.

Source: Clinical Case Reports - April 17, 2024 Category: General Medicine Authors: Polina Kostova, Guergana Petrova, Martin Shahid, Vera Papochieva, Dimitrinka Miteva, Ivelina Yordanova, Kossara Drenovska, Irena Bradinova, Camila K. Janniger, Robert A. Schwartz, Snejina Vassileva Tags: CASE REPORT Source Type: research