Successful Treatment of Two Rare Pediatric Keratinization Disorders with Secukinumab: Epidermolytic Ichthyosis and...

Pediatric erythroderma can arise from either inherited keratin defects or cytokine-driven inflammation, yet evidence for biologic therapies in these settings is limited. We report two rare pediatric cases successfully treated with secukinumab, an IL17A-targeted monoclonal antibody: (i) a 2-year-old boy with genetically confirmed epidermolytic ichthyosis (EI, KRT10 mutation:c.467G>A, p.Arg156His) refractory to conventional care, who achieved >60% improvement in erythema and scaling one week after a single off-label 150 mg subcutaneous secukinumab dose, with remission maintained for 12 months on monthly dosing; and (ii) an 11-year-old girl with coexistent Type III (juvenile) pityriasis rubra pilaris and acute generalized pustular psoriasis (PRP-GPP overlap) unresponsive to acitretin and methotrexate, who attained complete remission for 12 months following standard secukinumab induction (300mg weekly ×5) and maintenance every four weeks. These cases extend the potential utility of IL17A blockade beyond psoriasis vulgaris to both structural keratinopathies and inflammatory pustular dermatoses in children. While limited by the nature of a two-patient case series, these findings warrant prospective studies to clarify optimal dosing and long-term safety of IL-17A blockade in pediatric dermatology.