Secukinumab in Generalized Pustular Psoriasis.

Sir,

Generalized pustular psoriasis (GPP) is a potentially life threatening condition. Acute GPP, also called the von Zumbusch type, often starts abruptly, is associated with painful skin lesions, fever, and chills, and carries a significant morbidity in the absence of appropriate treatment. The disease can also run a chronic course.[1]

The first and second line treatments for GPP include acitretin, cyclosporine, methotrexate, and infliximab. Second line includes adalimumab, etanercept, PUVA, topical steroids, topical calcipotriene, and topical tacrolimus.[1]

Secukinumab is a new biologic approved in June 2015 by the US Food and Drug Administration for the treatment of adults with moderate-to-severe plaque psoriasis. It is a human monoclonal antibody that inhibits only IL-17A, leaving the IL-17F component for valuable immunity. There are a few case reports showing the efficacy of secukinumab in pustular psoriasis.[2345] We report a young lactating mother with GPP who responded dramatically after the first injection of secukinumab.

A 25-year-old married female was admitted in the emergency department with fever and erythematous scaly plaques studded with pustules for approximately 2 weeks. She had been on methotrexate 10 mg/week 2 years ago. She had plaque psoriasis for over 3 years and developed pustular psoriasis for the last 2 weeks. She was lactating and her baby was 4 months old. Her PASI (psoriasis area severity index) was 30.2 on admission [Figures 1 and 2]. Her investigations revealed leucocytosis, raised ESR, CRP, and elevated liver enzymes. Although her blood culture did not show any growth, she was started on IV antibiotics (cefoperazone sulbactam). Screening for tuberculosis and viral markers were negative. As she was of child bearing age, and liver enzymes were elevated, acitretin and methotrexate were not considered. We started her on cyclosporine 150 mg/day as she was lactating. After 1 week her fever subsided but there was no improvement in her skin lesions. Therefore, she was started on inj. secukinumab weekly 300 mg subcutaneous (week 0, 1, 2, 3, 4) and thereafter monthly for 3 months. The drug was continued for another 2 months at 150 mg/month after which it was stopped. Her pustules resolved after the first injection [Figures 3 and 4]. After almost 2 years of stopping the injections, the patient is still in remission and her child who is 2 years old now is normal.

Figure 1

Patient with pustular psoriasis showing erythematous scaly plaques studded with pustules over the chest

Figure 2

Erythematous scaly plaques studded with pustules over the right arm

Figure 3

Resolution of lesions over the chest 1 week after the first injection of secukinumab

Figure 4

Resolution of lesions over the right arm 1 week after the first injection of secukinumab

Diagnostic criteria for GPP include: (1) multiple sterile pustules on erythematous skin; (2) systemic symptoms such as fever and malaise; (3) the presence of histopathologically confirmed spongiform pustules; and (4) one or more of the following laboratory test alterations: leukocytosis with left shift, elevated erythrocyte sedimentation rate, elevated CRP, high antistreptolysin O antibody levels, elevation of IgG or IgA, hypoproteinemia, and hypocalcemia.[6]

In pustular psoriasis, IL-17 mRNA levels were found to be distinctively high in comparison with other clinical subtypes and healthy controls. The results of a previous study indicated that IL-17 and Th17 cells have an important role in pustular psoriasis and severe psoriasis.[7] A Japanese study in 12 patients with GPP showed marked improvement with inj. secukinumab. All the patients received secukinumab 150 mg subcutaneous at the baseline, week 1, 2, 3 and 4, and then every 4 weeks. Change in GPP severity from baseline was evaluated by clinical global impression (CGI). Nine of 12 patients showed “very much improvement.”[2] In this study, the authors noticed improvement in the erythema and pustules in the first week similar to our patient. There are 3 other case reports showing the successful use of inj. seckukinumab in GPP.[345] All patients showed a significant response at 3 weeks. Biologics are considered safe in lactation due to the minimal amounts of medication that are present in breast milk and they are also destroyed by infant gastric enzymes. Although actual data are primarily for anti-TNF agents, this safety profile is generalizable to the newer IL-12/23 and IL-17 inhibitors as well.[8]

We are reporting this case as there are very few case reports on secukinumab in pustular psoriasis and none from India. While some earlier reports have shown improvement at 3 weeks, our patient has shown PASI 90 after just 1 week. This case also highlights the safety of secukinumab in lactation. We conclude that secukinumab can be considered as a first line drug in the treatment of GPP.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.