The update of treatment strategies in pediatrics with generalized pustular psoriasis in China.

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.

Different Clinical Features of Pediatric Generalized Pustular Psoriasis in Patients with or without IL36RN Variants.

BACKGROUND: Generalized pustular psoriasis (GPP) is a rare and life-threatening autoinflammatory dermatological disease. IL36RN was reported to be the main pathogenetic basis for GPP. Only a few studies have reported on the correlation analysis of IL36RN variants and the phenotype of pediatric-onset GPP. METHODS: IL36RN was screened in 60 children diagnosed with GPP from January 2013 to January 2020, and their detailed clinical profiles were obtained. RESULTS: Forty-six out of 60 (76.67%) patients harbored IL36RN variants, and six IL36RN variants were found, of which two were novel variants that were reported for the first time. The frequency of IL36RN variants was significantly different among the subtypes of GPP (GPP with acrodermatitis continua of Hallopeau group (ACH), 100%; GPP without plaque psoriasis (PV) and ACH, 78.05%; GPP with PV group, 44.44%) (p = 0.018), while the percentage of IL36RN variants in the GPP with ACH group was higher than that in the GPP with PV group (p < 0.05). IL36RN variants were associated with a lower percentage of PV, longer length of hospitalization, and longer time to reach normal body temperature after treatment (p < 0.05). After treatment, marked responses, moderate responses, and no responses were recorded in 75.00%, 8.33%, and 16.67% of patients, respectively. No significant difference was observed during efficacy assessment in patients with or without IL36RN variants (χ2 = 1.122, p > 0.05). CONCLUSIONS: IL36RN variants are associated with GPP with ACH subtypes, an absence of concurrent PV, and a greater extent of severe inflammation. Acitretin was an effective treatment for patients in our study and mostly resulted in a marked response in our cohort.

Real-world data on the use of secukinumab and acitretin in pediatric generalized pustular psoriasis.

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.

Obesity and dyslipidemia in patients with psoriasis: A case-control study.

The aim of this study was to conduct a more comprehensive analysis of the association between psoriasis and abnormal lipid metabolism.The case-control study included 222 psoriatic patients and 445 non-psoriatic control patients matched for age and gender. Clinical parameters included age, gender, and body mass index (BMI). Serum lipid levels were recorded and included cholesterol (CHO), triglycerides (TG), low-density lipoprotein (LDL), high density lipoprotein (HDL), phospholipids (PLIP), free fatty acids (FFA), lipoprotein (a) [Lp(a)], and apolipoproteins (apoA1, apoB, and apoE). Statistical analysis was carried out through the IBM Statistical Package for the Social Studies version 23.0.Compared with controls, levels of BMI and the prevalence of obesity were significantly higher in psoriatic patients. The results revealed that when compared to controls, significant elevation of serum TG (P <.001) and Lp(a) (P = .022) was observed. Levels of HDL (P <.001) and apoA1 (P <.001) were significantly lower in psoriatic patients. There was no significant difference in CHO (P = .367), LDL (P = .400), apoB (P = .294), apoE (P = .05), PLIP (P = .931) and FFA (P = .554) between patients and controls. The levels of CHO, TG, PLIP, FFA, and apoE were positively correlated with BMI level.Dyslipidemia was more common in psoriatic patients, compared with non-psoriatic controls.

The functions of FoxO transcription factors in epithelial wound healing.

Re-epithelialisation is a complex process that involves the migration and proliferation of keratinocytes, as well as the production of cytokines and growth factors that affect wound healing. The precise mechanisms that control the tissue repair process remain poorly understood. Recent evidence indicates that FoxO transcription factors play a crucial role in wound healing. In mammals, different isoforms of FoxO, namely, FoxO1, FoxO3, FoxO4 and FoxO6, are present; however, FoxO1 and FoxO3 primarily function in epithelial wound healing. The functions of FoxO proteins in normal wound healing are opposite of those in diabetic wound healing. On the one hand, FoxO transcription factors promote the migration of keratinocytes through up-regulating the expression of transforming growth factor-beta and protecting keratinocytes from oxidative stress. On the other hand, FoxO transcription factors negatively regulate some genes that are needed for re-epithelialisation and keratinocyte migration. This review provides a summary of the functions of FoxO proteins in re-epithelialisation.

Abrupt generalized pustules in patients with rheumatoid arthritis and interstitial lung disease.

We report a case of a 30-year-old Chinese woman with rheumatoid arthritis and interstitial lung disease who abruptly developed generalized pustules and a high fever for 10 days. She had been taking oral prednisone, iguratimod and total glucosides of peony regularly for 5 months prior. In addition, she had taken metronidazole for 3 days 20 days prior which she had used before with no adverse reaction. She had no history of similar lesions and psoriasis. A biopsy of a pustule on the back showed spongiform pustule of Kogoj. She was suspected of having generalized pustular psoriasis or acute generalized exanthematous pustulosis. Finally, she was diagnosed with generalized pustular psoriasis (von Zumbusch type) considering the characteristics and clinical course of the rash. In addition to the above three drugs, systemic cyclosporin (5 mg/kg per day) was applied, and the lesions and fever resolved within the proceeding 2 months.