| Summary: | Summary Background Patients with severe autosomal recessive congenital ichthyosis (ARCI) show a T helper 17/interleukin 17 (Th17/IL17) skewing in their skin and serum, resembling the inflammatory profile of psoriatic patients. Secukinumab, an IL‐17A inhibitor, has shown clinical efficacy in patients with moderate‐to‐severe plaque psoriasis. Aims To test the clinical efficacy and safety of secukinumab in a paediatric patient with ATP‐binding cassette subfamily A member 12 deficiency‐related severe erythrodermic ARCI. Materials & Methods 6‐months therapeutic trial. During the first 4‐weeks induction period, the patient received weekly subcutaneous injections of 150 mg secukinumab (five injections in total). During the following 20‐weeks maintenance period, the patient was given a subcutaneous injection of 150 mg secukinumab every 4 weeks. Result & Discussion After the 6‐months therapy period, there was a 48% reduction from the baseline Ichthyosis‐Area‐Severity‐Index (‐Erythema/‐Scaling) score. The treatment was well tolerated. Moreover, cytokine analysis revealed a reduction of keratinocyte‐derived proinflammatory cytokines and an abrogation of Th17‐skewing during therapy. Conclusion Further studies are needed to evaluate the effects of the use of IL‐17A inhibition in ARCI patients.
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