Successful Treatment of Generalized Pustular Psoriasis with Secukinumab: A Report of Two Cases
Generalized pustular psoriasis (GPP) is a rare and severe form of psoriasis that poses significant challenges in management. Traditional treatment approaches, including systemic therapies such as acitretin, methotrexate, and cyclosporin, often come with notable side effects, limiting their clinical utility. Secukinumab, a fully human anti-interleukin (IL)-17A monoclonal antibody, has demonstrated efficacy in treating moderate to severe plaque-type psoriasis. However, there is limited real-world evidence on its effectiveness in treating GPP. This report details the successful treatment of two GPP cases with secukinumab, highlighting its potential as a therapeutic option.
Case 1 involved a 21-year-old Chinese man with a 4-month history of plaque-type psoriasis. His condition suddenly worsened following a flu-like episode, leading to a generalized pustular eruption affecting 86% of his body surface area (BSA). He presented with high fever (up to 41°C), severe itching, disseminated pustules primarily over the trunk and extremities, and pitting edema on his face and lower legs. His psoriasis area and severity index (PASI) was 32.5, and the dermatology life quality index (DLQI) was 15. Laboratory findings revealed neutrophilic leukocytosis, elevated liver enzymes, and increased C-reactive protein (CRP) levels, confirming a diagnosis of GPP.
The patient was started on secukinumab, administered subcutaneously at a dose of 300 mg once weekly for the first four weeks, followed by 300 mg every four weeks. Remarkably, within 8 hours of the first dose, his fever subsided to 36.5°C, and his general condition improved. Partial resolution of pustules and a marked reduction in erythema were observed within 48 hours. By the second dose, he achieved PASI-75, and by week 6, he reached PASI-90. Complete remission was observed at week 8, with a DLQI of 0, which was maintained until week 11.
Case 2 involved a 16-year-old Chinese girl with a history of plaque-type psoriasis since the age of 10. She developed GPP suddenly over seven days, with no obvious trigger. Her PASI was 37.8, and 42% of her BSA was affected. She was afebrile, and her DLQI was 17. Laboratory findings were unremarkable. She received the same secukinumab regimen as the first patient. Within four days of the first dose, her pustules resolved. By the first week, she achieved PASI-75, and complete remission was achieved at week 8, with a DLQI of 0, maintained until week 24.
The pathogenesis of GPP is closely linked to the recruitment of neutrophils and other inflammatory cells in the epidermis via the IL-36 and IL-36 receptor (IL-36R) pathway. The IL-36 receptor antagonist (IL-36RA), encoded by the IL-36RN gene, can block this pro-inflammatory signaling pathway. Additionally, the activation of the tumor necrosis factor (TNF)-a/IL-17/IL-22 axis exacerbates this reaction, with IL-17 playing a crucial role in the development of both plaque psoriasis and GPP.
A 52-week open-label phase 3 study in Japanese patients with GPP reported that 10 out of 12 patients (83%) showed significant improvement by week 16, with most experiencing the greatest improvement within three weeks and some achieving complete remission by week 12. Other studies have also highlighted the efficacy of secukinumab in treating GPP, including cases with IL-36RN mutations, suggesting its broad applicability across different GPP subtypes.
In this report, both patients achieved complete remission by week 8, which is earlier than the 12 weeks reported in previous studies where patients had failed prior therapies. This suggests that secukinumab may offer rapid and effective relief for GPP patients, even those who have not undergone extensive prior treatment.
In conclusion, secukinumab represents a promising therapeutic option for GPP, providing rapid symptom relief and significant improvement in quality of life. Further studies are warranted to confirm these findings and to explore the long-term efficacy and safety of secukinumab in treating GPP.
doi.org/10.1097/CM9.0000000000001244
Was this helpful?
0 / 0