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.

Systemic Methotrexate Treatment in 42 Children with Severe Plaque Psoriasis: A Retrospective Study in China.

BACKGROUND: The scientific evidence of methotrexate (MTX) in children with severe plaque psoriasis is scarce. OBJECTIVES: To retrospectively evaluate the efficacy and safety of oral MTX in children with severe plaque psoriasis in a single center in China. METHODS: We enrolled 42 children with severe plaque psoriasis who were administrated MTX. Efficacy was evaluated by the psoriasis area and severity index (PASI) score, physician global assessment (PGA) score, and body surface area (BSA) score. The Children's Dermatology Life Quality Index (CDLQI) score and safety data were recorded. RESULTS: Among 42 children (22 males, 20 females), the mean age was 11.2 years old. The initial weight-based dosage of oral MTX ranged from 0.1 to 0.3 mg/kg weekly. Overall, 80.6 and 47.2% of patients achieved PASI75 (at least 75% improvement from baseline in PASI score) and PASI90 (at least 90% improvement from baseline in PASI score) at week 12, respectively. 72.2% of patients achieved PGA 0/1 at week 12. BSA and PGA scores significantly decreased from baseline from week 4, accompanied by CDLQI score improvement from week 8. The steady effect of MTX could be reached at week 16. Elevated liver enzymes (28.6%) and infections (28.6%) were the most common side effects. Relapse was recorded in 9 (30.0%) of 30 patients, and the mean posttherapy disease-free interval was 7.2 months. CONCLUSIONS: MTX is an effective and safe option for children with severe plaque psoriasis with adequate monitoring.

Hypopigmented Mycosis Fungoides: A Clinical and Histopathology Analysis in 9 Children.

BACKGROUND: Hypopigmented mycosis fungoides (HMF) is an uncommon variant of mycosis fungoides. AIMS: To study the clinical and histopathology presentation in children with HMF. METHOD: We reviewed 9 children diagnosed with HMF. The clinical data were collected and analyzed. RESULT: Eight boys and 1 girl were included, with a median onset age of 7.4 year old and median age of diagnosis of 10.5 year old. Multiple hypopigmented patches were observed in all patients, and 5 patients exhibited multiple scaly erythema at the center of hypopigmented patches. Histopathology showed atypical lymphocytes with hyperchromatic, irregular, and cerebriform nuclei, infiltrated in the epidermis and dermis. Pautrier's microabscesses was noted in 6 of 9 patients, and papillary dermal fibroplasia was noted in 6 of 9 patients. CD8 predominance was detected in 4 of 6 patients. Four patients were simultaneously subjected to skin biopsy on hypopigmented patches and scaly erythema simultaneously. Compared with hypopigmented specimens, erythema biopsy detected deeper and denser infiltration of atypical lymphoid cells in 3 of 4 patients, higher CD4+/CD8+ ratio in 4 of 4 patients, more CD5 loss in 2 of 4 patients, and more CD7 loss in 2 of 4 patients. TCR gene monoclonal rearrangement was detected in 2 of 5 patients. Narrowband ultraviolet B phototherapy was applied in 7 patients. One of 7 patients achieved complete response, and 6 of 7 patients achieved partial response. No recurrence was noted with the median follow-up period of 6 months. CONCLUSION: HMF could occur in young patients, with indolent and benign course. HMF could gradually seem as scaly erythema based on hypopigmented patches. The histopathology indicated a more advanced stage of the scaly erythema lesions than hypopigmented patches.

Oral erythromycin in pityriasis lichenoides chronica and pityriasis lichenoides et varioliformis acuta.

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 observe the different treatment response to erythromycin in the pityriasis lichenoides chronica (PLC) and the pityriasis lichenoides et varioliformis acuta (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 =.05). 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, 90.9 % (n = 20) of patients responded. The response rate was higher in the PLEVA group than the PLC group (P = .004). 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.

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.

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.