PA08 The use of dupilumab for complex dermatoses: a case series

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Abstract Dupilumab is a biologic inhibitor of interleukin-4 and interkeukin-13 that is licensed for children aged ≥ 6 months with severe eczema. We present a case series of five children where dupilumab was used for atypical indications, or in complex cases where treatment options were limited. A child presented to dermatology aged 8 months with recalcitrant eczema since 2 weeks of age. She had coexistent immunodeficiency with severe generalized hypogammaglobulinaemia, and raised IgE. A dominant negative mutation in CARD11 was identified. She did not respond to topical and systemic steroids and ciclosporin. Dupilumab was initiated at age 7 years, with improvement of Eczema Area and Severity Index (EASI) score at 16 weeks from a baseline of 31.9 to 6.9. A patient with a background of heart transplant for idiopathic cardiomyopathy aged 2 years presented at age 6 years to dermatology with eczema. Her medications included azathioprine and tacrolimus. Response was lost to topical treatment in her early teenage years. With limited options and severe eczema, dupilumab was commenced. At 16 weeks, her EASI score had improved from 21.7 to 4.1. Her skin was clear on review at 6 months. Two siblings were referred to dermatology with atopic dermatitis. Both had comorbid mitochondrial disease requiring heart transplantation for dilated cardiomyopathy, and immunosuppression with ciclosporin. Genetic aetiology was not found on whole-exome sequencing. The elder sibling had recalcitrant eczema. Dupilumab was commenced age 15 years, with improvement of Children’s Dermatology Life Quality Index (CDLQI) from 17 to 5, and EASI score from 10.4 to 1.7 at 16 weeks. The younger sibling had recalcitrant, suberythrodermic eczema complicated by recurrent eczema herpeticum. Dupilumab was initiated at age 12 years, with an improvement of CDLQI from 26 to 3, and EASI score from 16.8 to 5.4 at 16 weeks. Both siblings’ eczema was severe in view of their significant concurrent immunosuppression, and both achieved dramatic improvement with dupilumab. A patient with harlequin ichthyosis had persistent erythematous and pruritic skin. Despite treatment with copious emollient, keratolytic agents, topical steroids and oral retinoids, she had ongoing issues with painful hyperkeratosis and fissuring. Dupilumab was commenced age 23 years, with dramatic improvement at 16 weeks in fissuring, skin tightness, scale and itch. DLQI had reduced from 10 at baseline to 3 at 16 weeks. Despite the complexity of each described case, with comorbid immunodeficiency, failure of other systemic medications and atypical indications, dupilumab worked well without harm or multidisciplinary team objection. It should be considered as a relatively safe and efficacious option in complex cases such as these.

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