Netherton Syndrome: Effect on Ichthyosis Linearis Circumflexa with Dupilumab

This case study evaluates the efficacy of Dupilumab in treating a 4-year-old girl with Netherton Syndrome (NS), specifically focusing on its impact on eczema and ichthyosis linearis circumflexa (ILC). Dupilumab treatment led to significant improvements in eczema, pruritus, and hair condition, with a more than 40% improvement in SCORAD, EASI, and CDLQI scores, and a substantial reduction in serum IgE levels. However, the treatment did not improve ILC, which persisted with recurrent flares. The study suggests that while Dupilumab effectively targets the Th2-driven pathways associated with eczema, it does not address the IL-17/IL-36 pathways implicated in ILC. This highlights the need for further research to develop targeted therapies for the ichthyotic lesions in NS.