|Year : 2022 | Volume
| Issue : 2 | Page : 159-161
Successful treatment of juvenile generalized pustular psoriasis with secukinumab monotherapy. A case report and review of the literature
Raghavendra Rao1, Krithiga Muralidharan1, Varsha M Shetty1, Sudhir U K. Nayak1, Vishwapriya Godkhindi2, Pallavi Hegde1
1 Department of Dermatology, Venereology and Leprology, Kasturba Medical College, Manipal Academy of Higher Education, Manipal, Karnataka, India
2 Department of Pathology, Venereology and Leprology, Kasturba Medical College, Manipal Academy of Higher Education, Manipal, Karnataka, India
|Date of Submission||29-Nov-2021|
|Date of Decision||24-Jan-2022|
|Date of Acceptance||24-Jan-2022|
|Date of Web Publication||30-Mar-2022|
Dr. Pallavi Hegde
Department of Dermatology, Venereology and Leprology, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal - 576 104, Karnataka
Source of Support: None, Conflict of Interest: None
Juvenile generalized pustular psoriasis (GPP) is an extremely rare variant of psoriasis, often accompanied by systemic symptoms. It adversely affects the psychosocial aspect of the child as well as their caregivers. Hence, there is a need for rapid control of the disease and induction of remission at the earliest possible time. In this report, we describe a case of a 15-year-old juvenile GPP patient with a history of multiple relapses, who responded dramatically to secukinumab monotherapy. This report is intended to highlight the use of safer biologics such as secukinumab in patients where other conventional drugs are not suitable to use.
Keywords: Juvenile psoriasis, pustular psoriasis, secukinumab
|How to cite this article:|
Rao R, Muralidharan K, Shetty VM, K. Nayak SU, Godkhindi V, Hegde P. Successful treatment of juvenile generalized pustular psoriasis with secukinumab monotherapy. A case report and review of the literature. Indian J Paediatr Dermatol 2022;23:159-61
|How to cite this URL:|
Rao R, Muralidharan K, Shetty VM, K. Nayak SU, Godkhindi V, Hegde P. Successful treatment of juvenile generalized pustular psoriasis with secukinumab monotherapy. A case report and review of the literature. Indian J Paediatr Dermatol [serial online] 2022 [cited 2022 May 20];23:159-61. Available from: https://www.ijpd.in/text.asp?2022/23/2/159/341466
| Introduction|| |
Juvenile generalized pustular psoriasis (GPP) is an extremely rare variant of psoriasis accounting for 0.4%–13% of pediatric psoriasis. It is thought to be more common among females; clinically, it is characterized by extensive sheets of erythema covered with pinhead-sized pustules which may fuse to form a lake of pustules. It is often accompanied by systemic symptoms (fever, malaise, and arthralgia) and laboratory disturbances (neutrophilia, liver test abnormalities, and elevated acute phase reactants). Although considered more benign disease compared to adults, juvenile GPP may adversely impact the psychosocial development and scholastic performance of the affected child, besides affecting the quality of life among the caregivers. Hence, it is imperative for clinicians to control the disease and induce remission at the earliest possible time. The treatment guidelines based on randomized control trials (RCT) do not exist for children; most of the recommendations are based on the case reports and/or case series. In this report, we describe a case of juvenile GPP successfully treated with secukinumab monotherapy.
| Case Report|| |
A 15-year-old female, a known case of psoriasis vulgaris for the past 7 years, presented with generalized pustular lesions for the past 3 weeks. She admitted her noncompliance to therapy leading to repeated cycles of remission and relapse in the past. The present episode started as plaque type of lesions 3 months ago, for which she was using topical corticosteroid (TCS). She abruptly stopped the application of TCS; within a span of the next 72 h, she developed multiple tiny pustules all over the body associated with burning pain. She was obese (body mass index of 45.9, >99th percentile) and had bilateral pedal edema. Cutaneous examination revealed diffuse sheets of erythema studded with pinhead-sized pustules and extensive desquamation, predominantly over the upper part of the body covering up to 70% body surface area (BSA) [Figure 1]a and [Figure 1]b. Erythematous plaques with classical psoriatic morphology were observed on the thigh, legs, and scalp. There was yellowish discoloration of the nail plate with subungual pustulation in few fingernails. Hematological and biochemical tests revealed leukocytosis, increased erythrocyte sedimentation rate, hypoproteinemia, and normal calcium levels. Biopsy from the representative lesion was consistent with the diagnosis of pustular psoriasis [[Figure 1] in the Supplement]. Various treatment options were discussed with the patient and the parents; in view of the previous history of intolerance to methotrexate (MTX) and apprehension about the side effect profile of systemic corticosteroids, cyclosporine, and acitretin, she was offered and consented to take secukinumab therapy. After negative sensitivity test, she was administered secukinumab (150 mg) subcutaneously after the workup for latent tuberculosis (a normal chest X-ray and a negative interferon-gamma release assay). She responded dramatically to the therapy and after two doses of secukinumab at weekly intervals, all pustules resolved and her skin returned to normal state [Figure 1]c and [Figure 1]d. She tolerated the treatment well. The treatment was continued at weekly intervals; after the fifth dose, she was maintained with monthly injections. At the time of writing (6 months since the initiation of the treatment), she did not develop the relapse of the disease.
|Figure 1: At the time of presentation, diffuse sheets of erythema studded with pinhead-sized pustules (a). and prominent desquamation predominantly over the chest and back were observed (b). (c and d) Near-complete resolution of the lesions after 2 doses (150 mg each) of secukinumab injections|
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| Discussion|| |
Various systemic therapies that have been used to treat GPP in children include MTX, oral retinoids, cyclosporine A (CyA), phototherapy, and biologics. Not all patients might respond to monotherapy, and frequently, a combination of systemic therapies is required to achieve the remission as observed in the previous reports. MTX and cyclosporine are generally preferred first-line therapies; our patient was intolerant to MTX. Although CyA has a rapid onset of action enabling faster disease control, it is not suitable as a maintenance therapy due to potential toxicities. Hence, one needs to choose a safer drug to maintain the remission. Biologics are generally considered for severe, recalcitrant, and difficult to treat pediatric GPP with the aim of achieving long-term remission. Tumor necrosis factor inhibitors (TNFi) have been widely used to treat different types of psoriasis in adults. Etanercept is the first TNFi that is approved by the food and drug administration (FDA) for pediatric plaque psoriasis. Adalimumab is approved for similar indications in few European countries. Both these biologics are demonstrated to have good efficacy in pediatric GPP as well, including few nonresponders to conventional systemic agents. Infliximab is generally reserved as a second-line biologic for pediatric patients with GPP in whom rapid disease control is required.
Secukinumab is a recombinant fully human monoclonal immunoglobulin G1 antibody that blocks interleukin 17A (IL17A) which plays a crucial role in the immunopathogenesis of psoriasis. The safety and efficacy of secukinumab in pediatric chronic plaque psoriasis have been established. Recently, both the European Commission and FDA have approved secukinumab for use in pediatric patients (aged >6 years) with moderate-to-severe plaque psoriasis who requires systemic therapy., The safety and efficacy of secukinumab have been demonstrated in adult GPP patients in a multicenter study from Japan. However, its use in pediatric GPP remains off-label and is limited to care reports and case series [Table 1].,,, Authors have reported the utility of secukinumab in pediatric GPP who showed the deficiency of IL-36 receptor antagonist (DITRA) due to mutations in IL36RN in the Taiwan, Chinese, and Japanese population.,, However, we could not perform mutation analysis due to the lack of facility.
|Table 1: Various Juvenile generalized pustular psoriasis cases reported in the literature treated with secukinumab|
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We chose to treat our patient with secukinumab considering the well-established efficacy and reassuring safety profile of this drug in the pediatric population, convenient dosing, less stringent laboratory monitoring, and convenience of day care treatment. However, the RCTs are still awaited to collect sufficient data and to recommend their use in the pediatric population as first-line agents.
Declaration of consent
The authors certify that they have obtained all appropriate consent forms, duly signed by the parent(s) of the patient. In the form the parent(s) has/have given his/her/their consent for the images and other clinical information of their child to be reported in the journal. The parents understand that the names and initials of their child will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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