Secukinumab monotherapy successfully treated severe refractory type V (atypical juvenile) pityriasis rubra pilaris: A case report and literature review

Abstract

Pityriasis rubra pilaris (PRP) is a rare heterogeneous group of papulosquamous inflammatory disorders with unknown etiology. PRP is often resistant to many conventional therapies which has made more challenging on treatment. More recently, several studies have shown encouraging clinical results of secukinumab in the treatment of PRP in adult, but no studies have explored its effects in children. We herein report a 7-year-old boy with severe type V PRP responded rapidly to secukinumab monotherapy (150 mg once weekly) when conventional therapies have failed. The patient showed rapid and dramatic improvement of erythema, palmoplantar hyperkeratosis, scaling, and itching within only 5 weeks, with no adverse effects. Secukinumab could be considered as a treatment option for refractory PRP in children, as recently reported in adult. K E Y W O R D S monotherapy, pityriasis rubra pilaris, secukinumab 1 | INTRODUCTION Pityriasis rubra pilaris (PRP) is an uncommon papulosquamous inflammatory disorder characterized by red-orange scaling erythema, follicular keratotic papules, and palmoplantar hyperkeratosis. 1 According to age at onset, clinical features, and prognosis, PRP can been subdivided into five clinical types: adult-onset PRP (classical, type I; atypical, type II) and juvenile-onset PRP (classical, type III; circumscribed, type IV; atypical, type V). 2 Type V PRP is clinically similar to type II PRP except for different onset ages, which are both typified by ichthyosiform features and usually chronic intractable, accounting, respectively, for about 5% of all cases. 1 The therapy in children is sometimes based on research and clinical experience from adults. More recently, secukinumab, an anti-IL-17A monoclonal antibody, has been showed promising results in small series and case reports in adult PRP. In this study, we present a 7-year-old boy with severe atypical juvenile PRP (type V) successfully treated with secukinumab alone who had an unsatisfactory response to first-and second-line treatment, and review all relevant cases reported in the English literature. 2 | CASE REPORT A 7-year-old Chinese boy presented with generalized erythematous plaques and scaly eruption which started on head and trunk during first month after the birth and progressed to involve his entire body. He was diagnosed as atopic dermatitis and generalized pustular psoriasis by other hospitals, and has tried different treatments such as topical corticosteroids, calcineurin inhibitors, systemic methotrexate, and acitretin without any significant benefit. Physical examination revealed confluent ichthyosiform erythematous eruption all over his body with a large amount of scales, both upper eyelids welling and ectropion with incomplete closure, conjunctiva congestion, radial wrinkles around his month, thick palmoplantar Jing-yao Liang and Rui-xian Ye have contributed equally to this study.

CARD14‐associated papulosquamous eruption (CAPE) in pediatric patients: Three additional cases and review of the literature

“…9 Given the activation of this IL-23/Th17 pathway with CARD14 mutations, a better therapeutic response to ustekinumab versus TNFα inhibition or broad immunosuppressants is not surprising. Furthermore, recognition that lesional skin in PRP shows activation of the IL-23/Th17 pathway, even without CARD14 mutation, [10][11][12][13][14] has led to successful intervention with ustekinumab 15,16 and the IL-17 pathway inhibitors secukinumab, 17,18 ixekizumab, 19 and brodalumab. 20…”

Successful treatment of severe pityriasis rubra pilaris with secukinumab in a 3-year-old boy

“…2,3 Although there have been numerous report of secukinumab successfully treating PRP, most of these cases have involved adults, with only one paediatric case, a 7year-old boy with Type V PRP. 4 Thus, to our knowledge, this first report that the IL-17A inhibitor secukinumab was effective in treating a child with severe Type III PRP. Although Type III PRP is often self-limiting, our patient's rash progressed rapidly with severe itching, even leading to the development of eyelid ectropion and erythroderma.…”

Pityriasis Rubra Pilar: Case Report and Literature Review

“…22 -25 Promising results have been reported with the use of immunobiologicals, including Anti-TNF agents (Infliximab, Etanercept and Adalimumab); anti-IL 12/ IL 23 (risankizumab) and ILA 17 agents (Brodalumab, Guselkumab, Ustekinumab, Ixekizumab and Secukinumab). [25][26][27][28][29][30][31][32][33][34][35] Despite the increasing indications for the use of immunobiologicals in this pathology, the intrinsic risks of their use must be considered, due to the immunological modulation/ suppression activity, which can cause a greater risk of developing serious infections and malignant diseases, like lymphomas. 36 While the exact mechanism of action of MTX in inflammatory skin diseases like PRP remains unclear, it presumably depends primarily on two different effects.…”