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pustular psoriasis, ixekizumab, biologic, pediatric dermatology, psoriasis
Introduction: Generalized pustular psoriasis (GPP) is a rare, severe variant of psoriasis that is uncommon in pediatric patients and can be refractory to many therapies. Ixekizumab, a monoclonal antibody that selectively inhibits interleukin 17-A, has been reported as safe and efficacious in patients with GPP, though minimal data exists on its use in the pediatric population.
Case presentation: A 17-year-old female with history of alopecia areata and pustular psoriasis, on ustekinumab, was admitted for a severe pustular psoriasis flare with systemic symptoms including fever and tachycardia which progressed to erythroderma and required vasopressor support. After minimal improvement with 24 hours on broad-spectrum antibiotics, she received cyclosporine 5mg/kg/day. On day three of admission, and after two days of cyclosporine with minimal improvement, she received a 160 mg loading dose of ixekizumab. She defervesced and transferred out of the ICU within 24 hours. She was successfully weaned off cyclosporine after her second dose of ixekizumab. She experienced no adverse reactions.
Discussion: Generalized pustular psoriasis is less common and often more severe than plaque psoriasis, associated with increased morbidity and mortality in both pediatric and adult patients. Clinical trials and case series have reported rapid and sustained improvement in patients with pustular psoriasis refractory to other therapies, though there is little data on the pediatric population. This case demonstrates the rapid efficacy of ixekizumab for severe erythrodermic pustular psoriasis in a pediatric patient, highlighting its use not only for refractory disease, but also as a rescue therapy in an emergent setting.
2. Wang WM , Jin HZ. Biologics in the treatment of pustular psoriasis. Expert Opin Drug Saf 2020;19:969-80.
3. Hoegler KM, John AM, Handler MZ , Schwartz RA. Generalized pustular psoriasis: a review and update on treatment. J Eur Acad Dermatol Venereol 2018;32:1645-51.
4. Marrakchi S, Guigue P, Renshaw BR, Puel A, Pei XY, Fraitag S et al. Interleukin-36-receptor antagonist deficiency and generalized pustular psoriasis. N Engl J Med 2011;365:620-8.
5. Wang S, Xie Z , Shen Z. Serum procalcitonin and C-reactive protein in the evaluation of bacterial infection in generalized pustular psoriasis. An Bras Dermatol 2019;94:542-8.
6. Okubo Y, Mabuchi T, Iwatsuki K, Elmaraghy H, Torisu-Itakura H, Morisaki Y et al. Long-term efficacy and safety of ixekizumab in Japanese patients with erythrodermic or generalized pustular psoriasis: subgroup analyses of an open-label, phase 3 study (UNCOVER-J). J Eur Acad Dermatol Venereol 2019;33:325-32.
7. Nagata M, Kamata M, Fukaya S, Hayashi K, Fukuyasu A, Tanaka T et al. Real-world single-center experience with 10 cases of generalized pustular psoriasis successfully treated with ixekizumab. J Am Acad Dermatol 2020;82:758-61.
8. Saeki H, Nakagawa H, Nakajo K, Ishii T, Morisaki Y, Aoki T et al. Efficacy and safety of ixekizumab treatment for Japanese patients with moderate to severe plaque psoriasis, erythrodermic psoriasis and generalized pustular psoriasis: Results from a 52-week, open-label, phase 3 study (UNCOVER-J). J Dermatol 2017;44:355-62.
9. Cordoro KM, Ucmak D, Hitraya-Low M, Rosenblum MD , Liao W. Response to Interleukin (IL)-17 Inhibition in an Adolescent With Severe Manifestations of IL-36 Receptor Antagonist Deficiency (DITRA). JAMA Dermatology 2017;153:106-8.
10. Molho-Pessach V, Alyan R, Gordon D, Jaradat H , Zlotogorski A. Secukinumab for the Treatment of Deficiency of Interleukin 36 Receptor Antagonist in an Adolescent. JAMA Dermatol 2017;153:473-5.
11. Yilmaz SB, Cicek N, Coskun M, Yegin O , Alpsoy E. Serum and tissue levels of IL-17 in different clinical subtypes of psoriasis. Arch Dermatol Res 2012;304:465-9.
12. Carrier Y, Ma HL, Ramon HE, Napierata L, Small C, O'Toole M et al. Inter-regulation of Th17 cytokines and the IL-36 cytokines in vitro and in vivo: implications in psoriasis pathogenesis. J Invest Dermatol 2011;131:2428-37.