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CASE REPORT
Year : 2021  |  Volume : 22  |  Issue : 2  |  Page : 160-161

Successful treatment of recalcitrant generalized pustular psoriasis in a young child with interleukin-36 receptor antagonist mutation with secukinumab monotherapy


Department of Paediatrics, Paediatric Dermatology Unit, Women and Children Hospital, Kuala Lumpur, Malaysia

Date of Submission11-Jan-2021
Date of Decision20-Jan-2021
Date of Acceptance27-Jan-2021
Date of Web Publication31-Mar-2021

Correspondence Address:
Henrietta Albela
Department of Paediatrics, Paediatric Dermatology Unit, Women and Children Hospital, Jalan Pahang 50586, Kuala Lumpur
Malaysia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpd.ijpd_3_21

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  Abstract 


Generalized pustular psoriasis (GPP) is a severe, life-threatening form of psoriasis. Deficiency of interleukin (IL)-36 receptor antagonist has been found in a subset of patients with GPP, which is usually more difficult to treat with conventional treatment. We report a case of a 4-year-old Malaysian-Chinese boy with GPP and homozygous mutation at c.115+6T>C within the interleukin-36 receptor antagonist (IL36RN) gene, who was treated successfully with off-label use of secukinumab monotherapy after failure of treatment with acitretin and cyclosporine A.

Keywords: Childhood, generalized pustular psoriasis, interleukin-36RN mutation, secukinumab


How to cite this article:
Albela H, Begum S, Leong KF. Successful treatment of recalcitrant generalized pustular psoriasis in a young child with interleukin-36 receptor antagonist mutation with secukinumab monotherapy. Indian J Paediatr Dermatol 2021;22:160-1

How to cite this URL:
Albela H, Begum S, Leong KF. Successful treatment of recalcitrant generalized pustular psoriasis in a young child with interleukin-36 receptor antagonist mutation with secukinumab monotherapy. Indian J Paediatr Dermatol [serial online] 2021 [cited 2021 Apr 18];22:160-1. Available from: https://www.ijpd.in/text.asp?2021/22/2/160/312837




  Introduction Top


GPP is a severe form of psoriasis with a subset of patient of having DITRA, which is usually more recalcitrant to treatment. The anti-IL-17A monoclonal antibody, secukinumab, has been recently shown to be efficacious among the adult GPP patients. However, its evidence for use in the paediatric GPP population remains lacking.


  Case Report Top


A 4-year-8-month-old Malaysian-Chinese boy presented with generalized pustular lesions since 6 months of age. Skin biopsy done during infancy confirmed the diagnosis of generalized pustular psoriasis (GPP), and genetic analysis done revealed homozygous mutation within the interleukin-36 receptor antagonist (IL36RN) gene at position c.115+6T>C. He was treated with oral acitretin since 4 months of age, along with intermittent course of low-dose prednisolone. His condition remains stable with acitretin (dose of up to 1 mg/kg), however, he had multiple episodes of GPP flare due to infections. Oral cyclosporine A (dose of up to 4.5 mg/kg/day) was added to the treatment regimen for a duration of up to 4 months but was not effective in achieving remission. Treatment with secukinumab was offered as a treatment option to which parents agreed and signed off-label use consent. Six doses of secukinumab have been given to date, with a dose of 150 mg each time. The first two doses were given 1 week apart, with the third dose being 1 month apart, and the following doses were 2 months apart. No other systemic therapy was given concurrently. Pustular lesions cleared up within few days of the first dose of secukinumab. His modified GPP-Psoriasis Area and Severity Index Score was reduced by almost 60% by 1 week after the first injection [Figure 1]a, [Figure 1]b, [Figure 1]c and achieved 100% clearance after two doses [Figure 1]c. He has remained in complete remission for 6 months since his first dose.
Figure 1: Serial skin findings for our patient. Severe pustular psoriasis flare with widespread erythema and erythema (Figure 1a). 90% skin clearance with residual mild erythema and pustulation, 1 week after the first secukinumab injection (Figure 1b). Complete clearance with maintained remission after 6 months of treatment (Figure 1c)

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  Discussion Top


GPP is a rare, severe, potentially life-threatening variant of psoriasis which is defined by primary, sterile pustules, with or without systemic inflammation, including fever, chills, and leukocytosis.[1] A study among the Chinese population demonstrated that patients with GPP subtype exhibited the highest mutation rate of IL36RN, with the c.115+6T>C mutation being the most common variant.[2] Treatment for GPP among young children is challenging as there is paucity in randomized controlled trials and standardized guidelines.

The most commonly used systemic medication as first line to control pediatric GPP include acitretin, cyclosporine A, and methotrexate which have a comparable short-term effect, but multiple interventions may be required to control this condition for long-term management.[3],[4] The anti-interleukin-17A monoclonal antibody, secukinumab, has been shown to have rapid improvement in erythema and pustules with sustained efficacy up to 1 year in adult GPP patients.[4] Although secukinumab has been recently approved by the European Commission as first-line biologics for moderate-to-severe plaque psoriasis in pediatric patients aged 6–18 years old, its use in GPP, on the other hand, remains unknown. There are only a few reported cases on successful use of secukinumab in the pediatric age group for GPP, one of which is a 6-year-old Taiwanese boy who, similar to our case, also achieved remission with secukinumab monotherapy with a similar dosage regimen of 150 mg/injection.[5] However, in our case, the frequency of the injection was prolonged to 2 months after the first two doses as the patient was able to maintain remission without any side effects observed. Our case demonstrates a significant benefit of secukinumab for young children with GPP associated with IL36RN mutation. Further studies are needed to evaluate the efficacy and safety of secukinumab in GPP patients.

Acknowledgement

We would like to thank the Director of Health Malaysia for his permission to publish this article.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Navarini AA, Burden AD, Capon F, Mrowietz U, Puig L, Köks S, et al. European consensus statement on phenotypes of pustular psoriasis. J Eur Acad Dermatol Venereol 2017;31:1792-9.  Back to cited text no. 1
    
2.
Wang TS, Chiu HY, Hong JB, Chan CC, Lin SJ, Tsai TF. Correlation of IL36RN mutation with different clinical features of pustular psoriasis in Chinese patients. Arch Dermatol Res 2016;308:55-63.  Back to cited text no. 2
    
3.
Posso-De Los Rios CJ, Pope E, Lara-Corrales I. A systematic review of systemic medications for pustular psoriasis in pediatrics. Pediatr Dermatol 2014;31:430-9.  Back to cited text no. 3
    
4.
Imafuku S, Honma M, Okubo Y, Komine M, Ohtsuki M, Morita A, et al. Efficacy and safety of secukinumab in patients with generalized pustular psoriasis: A 52-week analysis from Phase III open-label multicenter Japanese study. J Dermatol 2016;43:1011-7.  Back to cited text no. 4
    
5.
Ho PH, Tsai TF. Successful treatment of refractory juvenile generalized pustular psoriasis with secukinumab monotherapy: A case report and review of published work. J Dermatol 2018;45:1353-6.  Back to cited text no. 5
    


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