Treatment with dupilumab improved pruritus severity in twins with Sjögren–Larsson syndrome, suggesting promising outcomes.
Sjögren–Larsson Syndrome (SLS), now termed ALDH3A2 ‐syndromic epidermal differentiation disorder (sEDD), is a rare genetic disorder marked by thickened skin, spasticity, and intellectual disability. Intractable pruritus is a nearly universal and debilitating feature of SLS that remains poorly managed by current therapies. We describe 4‐year‐old twin girls with genetically confirmed SLS who showed significant and lasting improvement in itch following treatment with dupilumab, a biologic targeting interleukin‐4 receptor signaling. Within 6 months, pruritus severity scores declined markedly, improving sleep and quality of life without adverse effects, supporting further investigation of dupilumab for SLS‐associated itch.
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Gallagher et al. (2025) studied this question.
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