Related Experiment Video
Updated: Jun 7, 2025
The Goeckerman Regimen for the Treatment of Moderate to Severe Psoriasis
Published on: July 11, 2013
Successful Treatment of Pediatric Generalized Pustular Psoriasis (GPP) with Spesolimab: 5 Case Reports and
Yunliu Chen1,2, Zhaoyang Wang1,2, Yuan Liang1,2
1Department of Dermatology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijng, People's Republic of China.
Insights
Spesolimab effectively treated generalized pustular psoriasis (GPP) in children aged 4-12, significantly reducing inflammation and symptoms. This study suggests spesolimab is a promising treatment option for pediatric GPP patients.
Area of Science:
- Dermatology
- Immunology
- Pediatrics
Background:
- Generalized pustular psoriasis (GPP) is a severe, rare, and potentially fatal inflammatory skin condition.
- Interleukin-36 (IL-36) is a critical therapeutic target in GPP.
- Spesolimab, an IL-36 receptor antagonist, shows efficacy and safety in adults, but pediatric data are limited.
Purpose of the Study:
- To evaluate the efficacy and safety of spesolimab in pediatric patients with GPP.
- To assess the impact of spesolimab on clinical disease severity and inflammatory markers in children.
Main Methods:
- Five pediatric patients (aged 4-12 years) with GPP received a single dose of spesolimab.
- Clinical outcomes were assessed using GPPGA, GPPASI, and JDA severity index.
- Plasma levels of IL-36 cytokines and other inflammatory markers (IL-17A, IL-17C, IFN-γ, TNF, IL-6, IL-8) were measured.
Main Results:
- All patients achieved significant clinical improvement within one week, with normalized GPPGA scores and reduced GPPASI.
- Statistically significant reductions in plasma levels of IL-36α, IL-36β, IL-17A, IL-17C, and IL-6 were observed.
- No recurrence was noted during 2-8 months follow-up, with a favorable safety profile.
Conclusions:
- Spesolimab demonstrates promising efficacy and safety in treating pediatric GPP.
- The findings support spesolimab as a potential therapeutic option for children aged 4-12 years with GPP.
Background:
Generalized pustular psoriasis (GPP) is a rare, severe, and potentially life-threatening inflammatory cutaneous disease. IL-36 is a key treatment target in GPP. Spesolimab, a humanized monoclonal antibody of the IL-36 receptor, has demonstrated a good efficacy and a favorable safety profile in adults with GPP. However, data on its use in children are scarce.
Methods:
We treated patients aged 4-12 years with GPP with a single dose of spesolimab. The Generalized Pustular Psoriasis Physician Global Assessment (GPPGA) total score, GPPGA pustulation sub-score, Generalized Pustular Psoriasis Area and Severity Index (GPPASI), and the Japanese Dermatological Association severity index for GPP were evaluated. The levels of IL-36α, IL-36β, and IL-36γ were detected by the magnetic bead-based immunoassays, and the levels of IL-17A, IL-17C, IFN-γ, TNF, IL-6, and IL-8 were measured by the Olink proximity extension assay technology.
Results:
We included five patients (four boys and one girl) with a median age was 6.9 years old (range: 4.8 to 10.6 years), and a median age of onset of 1.7 years (range: 3 months-10 years and 5 months). After 1 week of spesolimab administration, all patients had a total GPPGA score of 0/1 and pustulation subscore of 0, all patients had a GPPASI of 50, and four patients had a GPPASI of 75. Meanwhile, plasma levels of IL-36α, IL-36β, IL-36γ, IL-17A, IL-17C, IFN-γ, TNF, IL-6, IL-8 all decreased, and those of IL-36α, IL-36β, IL-17A, IL-17C, and IL-6 were statistically significant. There was no recurrence after 2 to 8 months of treatment. No other adverse event was recorded apart from one patient who experienced an upper respiratory infection in the first week.
Conclusion:
Spesolimab might be a prospective option for children aged 4 to 12 years.
