Yunliu Chen

Background: CARD14-associated papulosquamous eruption (CAPE) is a spectrum of disease exhibited by patients with CARD14 mutations, which are rare and have a wide variety of clinical manifestations. Patients usually have limited response to traditional therapies. Methods: We retrospectively analyzed a case series of 8 patients with CAPE in China. Whole-exome sequencing (WES) was performed in all patients to identify the mutation type. Three patients received the treatment of secukinumab with a 52-week follow-up period. They achieved 84.6%, 76.9%, and 68.8% improvement in PASI score, respectively. Results: The study identified three new variants in CARD14 that had not been previously reported: c.392_397del, c.391_392delinsTT, and c.-280C>T. Three patients with different clinical manifestations showed good response to secukinumab. Conclusion: The mutation types in CARD14-associated papulosquamous eruption were various. IL-17A inhibitors, such as secukinumab, can be an alternative treatment option for pediatric patients with CAPE.

Background: Obesity is a prevalent comorbidity in children with pediatric psoriasis and is known to influence treatment outcomes in adults. In China, secukinumab, an anti‐IL‐17A biologic, is available for treating pediatric psoriasis. However, the impact of obesity on the effectiveness of secukinumab in children remains unclear. Objective: To evaluate the effect of obesity on the clinical response to secukinumab in pediatric patients with plaque psoriasis. Methods: This retrospective cohort study included 70 children with plaque psoriasis treated with secukinumab for at least 52 weeks. Data on demographics, body mass index (BMI), disease duration, prior systemic therapy, and disease severity were collected. Patients were stratified by BMI into a nonobese group (normal: < 85th percentile; overweight: 85th–< 95th percentile) and an obese group (≥ 95th percentile) based on age‐ and sex‐specific percentiles from national pediatric references. Treatment efficacy, measured by PASI100, PASI90, PASI75, and Investigator’s Global Assessment (IGA) 0/1 responses, was assessed at baseline and Weeks 4, 12, 36, and 52, when available. Mixed‐effects models and generalized estimating equations were used to analyze the impact of BMI or BMI status on treatment efficacy. Results: Of the 70 children included, 70.5% and 80.3% achieved PASI100 and PASI90 at weeks 24, respectively, with efficacy sustained through 52 weeks. Nonobese children had significantly higher PASI100 responses compared to obese children at Weeks 12 (69.8% vs. 35.7%) and 24 (76.6% vs. 50.0%, both p < 0.05). After adjusting for confounders, obesity was associated with a reduced likelihood of achieving PASI100 (OR = 0.32, 95% CI 0.13–0.81) and PASI90 (OR = 0.23, 95% CI 0.06–0.83). Higher BMI independently reduced the odds of achieving PASI100 (OR = 0.90, 95% CI 0.81–1.00, p = 0.043). Conclusion: Secukinumab is effective in treating children with plaque psoriasis; however, obesity and higher BMI negatively impact clinical outcomes.

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.

Generalized pustular psoriasis (GPP) is a severe subtype of psoriasis, commonly combined with systemic inflammation. Gene mutations have been found to be associated with GPP and vary by ethnicity. Systemic treatments are usually required for the severity and potential complications of GPP. However, there is no common consensus in China, especially among pediatric patients, whose data are scarce. Acitretin, methotrexate, and cyclosporine are widely used in pediatrics with GPP, while the adverse effects should be highlighted. The emergence of different biological agents brings us into a new era. This article discusses the genetic background of Chinese patients and demonstrates the evidence of treatment in pediatrics with GPP.

To evaluate the outcomes of secukinumab and acitretin use in children with generalized pustular psoriasis (GPP), we compared the efficacy and adverse events of secukinumab in 20 children and acitretin in 16 children with GPP from January 1, 2019, to January 30, 2022. Among the 20 patients treated with secukinumab, the average time for pustules to fade, temperature to normalize, and C‐reactive protein (CRP) to normalize was 3.83, 2.46, and 3.91 days, respectively. All patients recovered (Japanese Dermatological Association severity index score: 0/1) in 3 weeks. The adverse events were abnormal liver enzyme (10%), atopic dermatitis–like lesions (10%), herpes simplex (5%), and neutropenia (10%). For the patients treated with acitretin, the average time for pustules to fade, temperature to normalize, and CRP to normalize was 6, 6.14, and 8.73 days, respectively. The adverse events included mucocutaneous dryness (75%), dyslipidemia (37.5%), and abnormal liver enzyme (25%). These findings demonstrate that secukinumab has more favorable outcomes than acitretin, and secukinumab was well tolerated by the pediatric patients with GPP.

Pityriasis lichenoides (PL) is an uncommon cutaneous disorder. Oral erythromycin is proposed to be effective in treating the disease. Here we reported 16 pediatric patients with PL and systematically reviewed published literatures on erythromycin treatment response in pediatric PL patients, to observed the different treatment response to erythromycin in the PLC and the PLEVA groups. Sixteen patients, 8 with PLC and 8 with PLEVA, were treated with erythromycin. In the PLC group, 25% (n = 2) patients responded to erythromycin, while in the PLEVA group, 87.5% (n = 7) patients responded to erythromycin. The response rate was higher in the PLEVA group than the PLC group (P = 0.041). No side effect was reported in the 16 patients. A total of 34 children including 16 from our studies were included for further descriptive analysis, in which 12 had PLC and 22 had PLEVA. In the PLC group, 41.7% (n = 5) of patients responded to erythromycin while in the PLEVA group, 90.1% (n = 20) of patients responded. The response rate was higher in the PLEVA group than the PLC group (P = 0.001). In conclusion, erythromycin is effective and safe in the treatment of children with PL, and erythromycin was more effective in patients with PLEVA than PLC.