Abstract
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.
Highlights
Generalized pustular psoriasis (GPP) is a rare, severe variant of psoriasis that is uncommon in pediatric patients and can be refractory to many therapies
Generalized pustular psoriasis (GPP) is an uncommon life-threatening subtype of psoriasis which is characterized by widespread sterile pustules overlying erythematous plaques. 1, 2 GPP has a female predominance with a median age of onset during the fifth decade of life, though earlier onset is noted in patients with a family history of psoriasis or homozygous mutation of Interleukin (IL) 36RN.[3]
IL36RN and CARD14 are genes which encode proteins secreted by keratinocytes and are thought to contribute to the susceptibility of GPP. 1, 4 GPP accounts for 0.6% to 7% pediatric psoriasis cases, with a male predominance and onset between 3 and 16 years of age.[3]
Summary
Generalized pustular psoriasis (GPP) is an uncommon life-threatening subtype of psoriasis which is characterized by widespread sterile pustules overlying erythematous plaques. 1, 2 GPP has a female predominance with a median age of onset during the fifth decade of life, though earlier onset is noted in patients with a family history of psoriasis or homozygous mutation of Interleukin (IL) 36RN.[3]. She has a history of multiple pustular psoriasis flares in the past with known triggers including surgery and infections She has been recalcitrant to many systemic therapies including methotrexate, cyclosporine, prednisone, infliximab, etanercept, adalimumab, and ustekinumab. Laboratory studies were notable for leukocytosis with neutrophilic predominance, elevated sedimentation rate, C-reactive protein (CRP), elevated procalcitonin (PCT), elevated transaminases, and pyuria with trace bacteria on urinalysis These findings were concerning for both a robust inflammatory response as well as an infectious trigger. A skin biopsy was notable for a spongiotic dermatitis with a subcorneal pustule with a superficial and deep infiltrate including neutrophils and eosinophils consistent with pustular psoriasis Her skin examination progressed to erythroderma with generalized edema and lakes of pus most concentrated on the scalp though present diffusely (Figure 3). She was successfully weaned off cyclosporine after her second dose of ixekizumab, four weeks later, and has remained clear
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