Treatment with biologics may improve symptoms in subset of patients with congenital ichthyoses: Study

• The study included 98 patients with a mean age of 19.7 years.

• Both sexes were equally represented.

• Most of the patients (84.7%) had severe or very severe CI.

• Netherton syndrome (30%) and congenital ichthyosiform erythroderma (21.4%) were major subtypes.

Biologics Use:

• The major biologics used targeted IL-17, IL-12/IL-23, or the IL-4 receptor.

• Mean treatment duration was 22 ± 20.1 months.

Treatment Outcomes:

• The response rate was 45 (45.9%) patients, out of which 18 (18.3%) were good responders.

• Erythrodermic CI subsets showed the maximum response.

• IL-12/IL-23 and IL-4 receptor inhibitors were the most effective treatments in Netherton syndrome and CIE.

Comparative Analysis with Literature Data:

•The mean duration of the application of biologic (11.5 ± 8.5 months).

• The efficacy, as reported, was higher, at 85.7% responders, which may indicate the limitation of case reports.

Biologics have proved promising in the treatment of erythrodermic forms of congenital ichthyoses, including Netherton syndrome and CIE. This study has clearly established their possibilities as a transformative therapy, paving the path for clinical trials in further research involving managing such severe genetic disorders.

Reference:

Mazereeuw-Hautier, J., Granier Tournier, C., Hernandez-Martin, A., Milesi, S., Texier, H., Severino-Freire, M., Bellon, N., Bodemer, C., Gruber, R., Mahé, E., Morice Picard, F., Hannula-Jouppi, K., Murase, J. E., Barbarot, S., Cohen-Barak, E., Torres-Pradilla, M., Bruckner, A., Levy, M., Koh, M. J. A., … Paller, A. S. (2024). Biologics in congenital ichthyosis: are they effective? The British Journal of Dermatology. https://doi.org/10.1093/bjd/ljae420