Current guidelines recommend psoriatic patients with latent tuberculosis infection undergo chemoprophylaxis prior to initiating any biologic. However, clinical studies indicate that interleukin (IL) inhibitors may not increase the risk of tuberculosis reactivation. This review evaluates the safety in psoriatic patients with latent tuberculosis infection using IL inhibitors without chemoprophylaxis. PubMed and EMBASE were searched up to 1 November 2024 in accordance with PRISMA. Fifteen studies, including one safety analysis of a clinical trial, 2 case series, and 12 retrospective studies were analysed. The included studies reported a total of 837 cases: 179 patients were treated with secukinumab, 69 with ixekizumab, 8 with brodalumab, 539 with risankizumab, 22 with guselkumab, and 20 with tildrakizumab. Psoriatic patients with latent tuberculosis infection using an IL-12/23 inhibitor without chemoprophylaxis were not found in this review. Three of the 837 cases exhibited reactivation of tuberculosis. The reactivation rate is 0.78% among psoriatic patients with latent tuberculosis infection using IL-17 inhibitors, and 0.17% among those using IL-23 inhibitors. Our analysis shows that IL-17 and IL-23 inhibitors do not increase the risk of tuberculosis activation in psoriatic patients with latent tuberculosis infection. The impact of IL-12/23 inhibitors on tuberculosis reactivation among psoriatic patients with latent tuberculosis infection remains uncertain and requires further investigation.

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.

INTRODUCTION:Pediatric-onset generalized pustular psoriasis (PGPP) is a rare inflammatory disorder characterized by recurrent flares and challenging management. This study aimed to review the clinical features, flare patterns, and treatment options. METHODS:We analyzed PGPP patients diagnosed at Beijing Children's Hospital from 2006 to 2023 who had complete medical record. Data on clinical characteristics, flare numbers, and flare duration were collected through medical record and telephone survey. The flare frequency was calculated by dividing the flare numbers by the years in each age range. Flare duration was categorized as ≤4 weeks or >4 weeks. In addition, we investigated the factors associated with flare frequency in PGPP patients. RESULTS:A total of 109 patients with PGPP were included in the study, with a follow-up duration ranging from 1.90 to 22.80 years. The median age of onset was 6.00 years, with a higher prevalence in males (73, 66.97%). Most patients (55, 50.46%) experienced their first flare between the ages of 7 and 12 years. A total of 45 patients (41.28%) had history of psoriasis vulgaris, 16 (14.68%) had history of acrodermatitis continua of Hallopeau, and 4 (3.67%) had history of psoriatic arthritis. Respiratory infection was the most common trigger, affecting 35 patients (32.11% of cases). The annual flare frequency was 0.68. The frequency and duration of flares showed a decreasing trend with increasing age. Earlier onset age is associated with a higher flare frequency. Before 2020, conventional therapies were most commonly used, with acitretin used in 67 patients (61.47%). Following 2020, secukinumab became the most often used medication (44 patients, 40.37%). CONCLUSION:PGPP exhibits a trend toward flare decrease with age, yet recurrence remains common. Earlier onset age may be associated with higher flare frequency. At present, biologic agents are the main treatment. These findings provide new insights into PGPP.

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.

We report a patient who presented with erythematous plaques with scales distributed all over the body, following the lines of Blaschko. The patient was diagnosed clinically with inflammatory linear verrucous epidermal naevus. Deep next-generation sequencing of the lesion showed a somatic mutation in CARD14. The patient was treated with secukinumab and experienced significant improvement.

Generalized pustular psoriasis (GPP) is a severe inflammatory cutaneous disease characterized by widespread pustules, edema, erythema, fever, and systemic inflammation. Chinese data indicate that the prevalence and incidence of GPP follow a bimodal age distribution, with peaks in the 0–3 year age group and the 30–39 year age group.1 In the 0–3 year age group, the prevalence was 0.927 and the incidence rate was 0.742 per 100 000 population-years.1 Interleukin (IL)-36 plays an important role in GPP by activating nuclear factor-κB (NF-κB) and mitogen-activated protein kinase (MAPK) signal pathways.2 Chemokines (CXCL8, CXCL1, CXCL2, etc.), cytokines (IL-1β, tumor necrosis factor TNF-α, IL-6, IL-23, IL-17, etc.), and activated cells (e.g., keratinocyte, neutrophils, dendritic cells, etc.) are also involved.3 Acitretin, cyclosporine, methotrexate, and etanercept were recommended as first-line treatments for children with GPP in 2012 by the American National Psoriasis Foundation.4 However, recent findings suggest that biological agents targeting IL-36, TNF-α, IL-17, IL-23, or their receptors might be more promising options than conventional drugs.5 Clinical trials and case series have also demonstrated the superiority of biological agents in adult and older pediatrics with GPP, including spesolimab, etanercept, adalimumab, secukinumab, brodalumab, and others.6 However, these biological agents, have only been approved by the FDA for children aged 4 years or older, and there is still limited data on their use in GPP patients under 4 years old.7, 8 Here, we report on six children under the age of 4 years who were treated for GPP using etanercept from July 2021 to January 2023. The diagnosis of GPP was based on the criteria set by the Japanese Dermatological Association (JDA).9 A complete blood count, liver function test, kidney function test, antibody test for viral hepatitis B and C, T-SPOT.TB test, antinuclear antibody (ANA) test, and chest X-ray were conducted before initiating treatment with etanercept. These tests were repeated every six months following the initial administration of etanercept. The initial dose of etanercept was 0.8 mg/kg per week, which was gradually adjusted to every 10 days, every 2 weeks, and every 3 weeks as the patients achieved stable remission. The severity of GPP was assessed using the JDA severity index for GPP9 and Generalized Pustular Psoriasis Area and Severity Index (GPPASI). The primary efficacy endpoint was achieving a JDA severity index of 1 or 0 at week 4. The secondary efficacy endpoint was achieving a 75% reduction in baseline GPPASI scores (GPPASI 75) at week 4. Relapse was defined as an increase of 3 points or more on the JDA score. Six girls with GPP were recruited, with ages ranging from 3 to 47 months and onset ages ranging from 20 days to 17 months (Table 1). One patient (patient 2) was simultaneously diagnosed with Acrodermatitis continua of Hallopeau (ACH). Homozygous or compound heterozygous mutations of the IL-36RN gene were found in five patients. No variants were detected in the CARD14, IL-1RN, AP1S3, and MPO genes. Five patients had previously failed to respond to prednisone or acitretin (Table 1). At baseline, one patient was classified as mild, two as moderate, and three as severe based on the JDA score. After 4 weeks of treatment, all six patients achieved a JDA score of 1 and GPPASI 75 (Table 2). After 12 weeks of treatment, all six patients reached a JDA score of 0 and a 100% reduction in their baseline GPPASI scores. In addition, the response was maintained for 24 weeks. The median duration of etanercept treatment was 24 weeks (range: 24–60 weeks). One patient (Patient 2) switched to adalimumab after 24 weeks due to no further improvement in ACH lesions. She achieved almost complete clearance of the ACH lesions with adalimumab over an additional 24 weeks (Figure 1). Two patients (Patient 5 and Patient 6) were still receiving etanercept treatment. The remaining three patients were followed for 10–12 months. One patient (Patient 1) experienced a relapse at 4 months after discontinuing the drug and was subsequently re-administrated etanercept. However, the 16-week treatment did not yield satisfactory results. Adalimumab was then applied, resulting in a JDA score of 1 at 4 weeks, which was maintained for 16 weeks. During the treatment period, two patients (Patient 1 and Patient 3) experienced a transient increase in ANA titer, up to 1:100, without any related symptoms. No other adverse events were recorded. Etanercept is a widely used TNF receptor inhibitor for treating plaque psoriasis, with demonstrated long-term efficacy and safety in children aged 4 years and older.10 However, data on its use in children under 4 years old are limited. In this study, we observed rapid and sustained improvement of GPP in young children treated with etanercept, regardless of IL-36RN gene mutation status. During the 24–60 week treatment period, no children experienced opportunistic infections, tuberculosis, or demyelination events. Two patients had a transient increase in ANA titer. These findings suggest that etanercept may be effective and safe for children under 4 years old with GPP. Considering the adverse events and financial burden, the etanercept was gradually tapered and eventually discontinued upon achieving stable complete remission. However, in adults with GPP, Bellinato et al.11 reported a relapse rate of 62% in the group discontinuing etanercept, compared to only 9% in the group continuing treatment. Fortunately, the median time to relapse was 184 days, and 52% of patients experienced clinical benefit upon re-administration of etanercept.11 In our study, we attempted to extend the treatment interval upon achieving stable remission, resulting in no recurrence during treatment. During the long follow-up period (10–12 months) for three patients, only one experienced a relapse. Thus, tapering and discontinuing etanercept when patients achieve stable remission is worth considering. Unfortunately, the relapsed patient who did not experience satisfactory improvement upon re-administration of etanercept subsequently required a switch to adalimumab. The study was approved by the Beijing Children's Hospital Ethics Review Board ([2024]-E-087-R). Informed consent was obtained from all participants' parents. The authors declare no conflict of interest.

Summary Lipodystrophia centrifugalis abdominalis infantilis (LCAI) is a localised atrophic disease. It is characterised by a depression with a periphery of erythema present on the lower abdomen and/or the groin region, axilla or neck. The depressed lesions could enlarge centrifugally to the neighbouring areas. Ulceration is a rare manifestation of this disease. In this study, we reported a case of LCAI presented with recurrent giant ulcerations on the abdomen, and was eventually successfully treated with hydroxychloroquine and baritinib.

例1 男, 12岁10个月, 全身反复红斑、脓疱9年, 加重3个月于2020年12月入院。9年前无明显诱因全身出现弥漫性红斑、脓疱, 伴高热, 结合皮损病理表现诊断为脓疱型银屑病, 先后使用阿维A、环孢素、甲氨蝶呤及重组人Ⅱ型肿瘤坏死因子受体-抗体融合蛋白等治疗, 病情可缓解, 但仍反复发作。3个月前皮损复发, 2周前皮损突然加重, 伴持续性高热, 最高39.9 ℃。无药物过敏史, 个人史无特殊, 无银屑病家族史。入院体检:体温39 ℃, 身高143 cm, 体重34 kg, 心肺腹部等检查未见异常。皮肤科检查:躯干、四肢泛发性水肿性红斑, 上覆米粒大小脓疱, 融合形成脓湖, 伴黄色云片状鳞屑(图1A);束发征、地图舌均阳性;关节无肿胀, 活动自如。实验室检查:C反应蛋白77 mg/L(参考值:< 8 mg/L, 下同), 白细胞计数17.11 × 109/L(4 × 109/L ～ 10 × 109/L), 中性粒细胞计数3.15 × 109/L(1.4 × 109/L ～ 6.5 × 109/L);白蛋白45.4 g/L(35 ～ 55 g/L)。肝肾功能、乙肝病毒抗原抗体、丙肝病毒抗体、结核感染T细胞检测(T-SPOT)、胸部X线片筛查均未见异常。脓疱皮损处分泌物培养阴性, 血培养阴性。自身炎症性疾病相关基因检测:患儿IL-36RN基因存在c.115+6T>C纯合突变及c.227C>T杂合突变, 其中c.115+6T>C纯合突变分别来自父亲及母亲, c.227C>T杂合突变来自父亲。

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.

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.

Objective:To compare the efficacy and safety of biologics versus methotrexate in the treatment of severe pediatric plaque psoriasis.Methods:A retrospective matched case-control study was carried out. Twenty children with severe plaque psoriasis from Beijing Children′s Hospital, Capital Medical University from June 2016 to November 2021 were included in this study, and the patients treated with biologics (adalimumab or secukinumab) were matched with those treated with methotrexate at a ratio of 1∶1 according to the psoriasis area and severity index (PASI) score and age. PASI, physician′s global assessment (PGA) , and body surface area (BSA) scores were assessed at weeks 4, 8 and 12 after the start of treatment, and adverse drug reactions were recorded. Statistical analysis was mainly carried out by using Mann-Whitney U test, Fisher′s exact test and generalized estimating equations. Results:At weeks 4 and 8, the proportions of patients achieving PASI75 and PASI90 were significantly higher in the biologics group (PASI75: 7/10, 10/10, PASI90: 5/10, 9/10, respectively) than in the methotrexate group (PASI75: 1/10, 5/10, PASI90: 0, 1/10, respectively; all P < 0.05) , while there was no significant difference between the biologics group and methotrexate group at week 12 (PASI75: 10/10 vs. 8/10, PASI90: 9/10 vs. 4/10, both P > 0.05) . There were no significant differences in the PASI, BSA or PGA scores between the two groups at baseline (all P > 0.05) , while the biologics group showed significantly decreased PASI and BSA scores at weeks 4, 8 and 12, and significantly decreased PGA score at week 8 compared with the methotrexate group (PASI: Z = 2.50, 3.56, 2.63, respectively; BSA: Z = 2.87, 3.57, 2.40, respectively; PGA: Z = 2.81; all P<0.05) . Analysis of changes over time showed that the PASI, PGA and BSA scores in the biologics group significantly decreased at weeks 4, 8 and 12 compared with those at baseline (all P<0.01) ; the PASI and PGA scores significantly decreased at weeks 8 and 12 compared with the corresponding scores at week 4 (all P<0.05) ; however, there were no significant differences in the PASI, PGA or BSA scores between week 12 and 8 (all P>0.05) . In the methotrexate group, the PASI, PGA and BSA scores at weeks 4, 8 and 12 were all significantly lower than the corresponding scores at the previous adjacent time points (all P<0.05) . There was no significant difference in the incidence of adverse reactions between the two groups ( P = 0.650) , and no serious adverse reactions occurred in either group. The main adverse reaction was infection in the biologics group, while infection and elevation of transaminase levels were common in the methotrexate group. Conclusion:Biologics and methotrexate were both effective and safe for the treatment of severe pediatricplaque psoriasis, and biologics facilitated rapider achievement of PASI75 and PASI90 compared with methotrexate.

Australasian Journal of DermatologyEarly View RESEARCH LETTER Clinicopathological characteristics, treatment and follow-up of lipodystrophia centrifugalis abdominalis infantilis: A retrospective case series in China Xinrong Zhao MD, Xinrong Zhao MD orcid.org/0000-0003-2493-9031 Department of Dermatology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, ChinaSearch for more papers by this authorYuanxiang Liu MD, Yuanxiang Liu MD orcid.org/0000-0001-9622-7899 Department of Dermatology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, ChinaSearch for more papers by this authorXin Xiang MD, Xin Xiang MD Department of Dermatology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, ChinaSearch for more papers by this authorZhaoyang Wang MD, Zhaoyang Wang MD Department of Dermatology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, ChinaSearch for more papers by this authorYunliu Chen PhD, Yunliu Chen PhD orcid.org/0000-0003-2259-6442 Department of Dermatology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, ChinaSearch for more papers by this authorChaoyang Miao PhD, Chaoyang Miao PhD orcid.org/0000-0003-3454-9897 Department of Dermatology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, ChinaSearch for more papers by this authorZigang Xu PhD, Corresponding Author Zigang Xu PhD [email protected] Department of Dermatology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China Correspondence Zigang Xu, Department of Dermatology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, No. 56 Nanlishi Road, Xicheng District, Beijing 100045, China. Email: [email protected]Search for more papers by this author Xinrong Zhao MD, Xinrong Zhao MD orcid.org/0000-0003-2493-9031 Department of Dermatology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, ChinaSearch for more papers by this authorYuanxiang Liu MD, Yuanxiang Liu MD orcid.org/0000-0001-9622-7899 Department of Dermatology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, ChinaSearch for more papers by this authorXin Xiang MD, Xin Xiang MD Department of Dermatology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, ChinaSearch for more papers by this authorZhaoyang Wang MD, Zhaoyang Wang MD Department of Dermatology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, ChinaSearch for more papers by this authorYunliu Chen PhD, Yunliu Chen PhD orcid.org/0000-0003-2259-6442 Department of Dermatology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, ChinaSearch for more papers by this authorChaoyang Miao PhD, Chaoyang Miao PhD orcid.org/0000-0003-3454-9897 Department of Dermatology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, ChinaSearch for more papers by this authorZigang Xu PhD, Corresponding Author Zigang Xu PhD [email protected] Department of Dermatology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China Correspondence Zigang Xu, Department of Dermatology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, No. 56 Nanlishi Road, Xicheng District, Beijing 100045, China. Email: [email protected]Search for more papers by this author First published: 14 December 2023 https://doi.org/10.1111/ajd.14203Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onEmailFacebookTwitterLinkedInRedditWechat No abstract is available for this article. CONFLICT OF INTEREST STATEMENT None declared. REFERENCES 1Giam YC, Rajan VS, Hock OB. Lipodystrophia centrifugalis abdominalis infantilis. Br J Dermatol. 1982; 106(4): 461–464. 10.1111/j.1365-2133.1982.tb04541.x CASPubMedWeb of Science®Google Scholar 2Cheng JW, Lin ZT, Li JH. Progressive skin depressions in a Child's neck. JAMA Dermatol. 2022; 158(6): 687–688. 10.1001/jamadermatol.2022.0760 Web of Science®Google Scholar 3Higuchi T, Yamakage A, Tamura T, Miyachi Y. Lipodystrophia centrifugalis abdominalis infantilis occurring in the neck. Dermatology. 1994; 188(2): 142–144. 10.1159/000247120 CASWeb of Science®Google Scholar 4Chen YA, Hsu CK, Lee JY, Yang CC. Linear lupus panniculitis of the scalp presenting as alopecia along Blaschko's lines: a distinct variant of lupus panniculitis in east Asians? J Dermatol. 2012; 39(4): 385–388. 10.1111/j.1346-8138.2011.01455.x CASPubMedWeb of Science®Google Scholar 5Xu Q, Qiu Y, Lu Z, Yu H, Ling B, Li Y, et al. Centrifugal lipodystrophy on a spectrum with lupus erythematosus panniculitis in children and efficacy and safety of hydroxychloroquine: a clinicopathological study. 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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.

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.

Objective:To evaluate the effect of oral acitretin on the height and bone development of children.Methods:Clinical and imaging data were collected from 106 children receiving oral acitretin for at least 1 month in Department of Dermatology, Beijing Children′s Hospital from March 2007 to January 2021, and retrospectively analyzed. The main outcome measures were height and near-adult height. Multivariate logistic regression analysis was carried out to investigate relevant factors for short stature in children, and non-inferiority test was used to analyze the proximity of the actual height to target height of children who had reached near-adult height. The secondary outcome measures were bone age and epiphyseal closure. Wilcoxon signed-rank test was used to analyze differences in the value of bone age minus chronological age between the baseline and last follow-up, and the premature closure of epiphysis was also evaluated.Results:Among the 106 children, 62 were males and 44 were females; 84 were diagnosed with pustular psoriasis, 10 with psoriasis vulgaris, 11 with pityriasis rubra pilaris, and 1 with lupus miliaris disseminatus faciei. These children received oral acitretin at doses of <1 mg·kg -1·d -1 for 1 - 90 months. Among the 96 children aged under 18 years, 91 (94.8%) were of normal stature, and 5 (5.2%) were short in stature; among the 83 children receiving acitretin monotherapy, 81 (97.6%) were of normal stature, and 2 (2.4%) of short stature. Binary logistic regression analysis showed that the risk of short stature caused by acitretin combined with glucocorticoid therapy was 76.57 times higher than that of acitretin monotherapy ( OR = 77.57, 95% CI: 2.20 - 2 738.82, P = 0.017) , while the type of disease, gender, age at onset, age at initial treatment with acitretin, course of treatment, and average daily dose of acitretin did not significantly affect the stature of children ( P = 0.988, 0.214, 0.087, 0.078, 0.066, 0.350, respectively) . At the last follow-up visit, 13 children who had reached near-adult height were of normal stature, and the non-inferiority test showed that their near-adult height was not inferior to the target height (Satterthwaite = 0.23, P = 0.030) . Bone age was evaluated in 45 children at baseline and last follow-up visit, there was no significant difference in the value of bone age minus chronological age between the baseline and last follow-up ( Z = -0.85, P = 0.250) , and no patients experienced premature closure of epiphysis before and after the treatment. Conclusion:This study preliminarily revealed that oral acitretin at doses of <1 mg·kg -1·d -1 for less than 90 months might not significantly affect the height and bone development of children.

Objective:To analyze pathogenic mutations in a child with ankyloblepharon-ectodermal defects-cleft lip/palate syndrome.Methods:Clinical data were collected from a patient with ankyloblepharon-ectodermal defects-cleft lip/palate syndrome, and DNA was extracted from peripheral blood samples from the patient and his parents. High-throughput sequencing was performed in the patient by using a gene panel targeting hereditary skin diseases, aiming to determine sites of disease-causing gene mutations. Then, Sanger sequencing was conducted to bidirectionally verify the mutations in the patient and his parents.Results:The male patient aged 3 years and 9 months, and presented with extensive erythema, scales, erosions as well as repeated infections and erosions of the scalp after birth. Reticulated hyper- and hypopigmented patches and scars left on the trunk and limbs after healing of erosions. Physical examination also showed sparse scalp hair, absence of most eyebrows and eyelashes, cleft palate, dysplastic teeth, dystrophic finger and toe nails, and deformed ears without ankyloblepharon. Genetic testing of the patient showed a novel heterozygous missense mutation c.1790T>A (p.Ile597Asn) in the TP63 gene, which had not been reported previously and was rated as pathogenic according to the American College of Medical Genetics and Genomics guidelines. This mutation was not identified in either of his parents.Conclusion:The novel heterozygous missense mutation c.1790T>A in the TP63 gene probably contributes to ankyloblepharon-ectodermal defects-cleft lip/palate syndrome in the patient, which expands genotypic and phenotypic spectrum of this disease.

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.

Dear Editor, Paediatric acrodermatitis continua of Hallopeau (ACH) is a rare, chronic, usually localized type of pustular psoriasis manifesting as sterile pustules affecting the nail apparatus. However, ACH is not only restricted to the nail apparatus, but can also spread to a generalized form, known as generalized pustular psoriasis (GPP). Our previous study showed that IL36RN mutations might be an underlying cause of paediatric ACH. ACH is invariably recalcitrant to traditional systemic drugs, although biologics are promising for its management. Adalimumab is a humanized monoclonal antibody selective for tumour necrosis factor (TNF)-a, and several case reports have shown its effectiveness in adults with ACH. We report a case series that illustrates the efficacy of adalimumab on children diagnosed with ACH. We retrospectively analysed a cohort of eight patients (three boys and five girls) with ACH who were treated with adalimumab during the period August 2020 to May 2021. A weight-based dose of adalimumab was administered through subcutaneous injection every other week, starting from 1 week after the initial administration. The effectiveness of the therapy was evaluated by the Clinical Global Impression (CGI) score. The primary efficacy endpoint was the proportion of patients who achieved a CGI score of ‘very much improved’ or ‘much improved’ at Week 12. Information about patient demographics, clinical characteristics, previous systemic treatments, IL36RN mutation and efficacy of adalimumab was collected (Table 1). The mean age of the patients was 10.3 3.7 years (range 6.3–15.9 years). ACH preceded the onset of GPP in five patients (62.5%). The mean age of onset was 4.2 3.9 years for ACH and 5.3 4.3 years for GPP. Three patients (37.5%) were simultaneously diagnosed with GPP, three with localized pustular psoriasis (LPP) and one with inverse psoriasis. Arthritis was identified in five cases (62.5%), with two of the five (40.0%) showing enthesitis, two showing bone destruction and one showing rough joint surface. IL36RN screening was performed in five cases (62.5%) and homozygous mutations or compound heterozygous mutations were found in all cases. ACH is invariably recalcitrant to traditional systemic drugs and biologics are promising for its management. All patients had been resistant to acitretin, methotrexate, ciclosporin, leflunomide or colchicine; etanercept had been shown to T b le 1 D em o g ra p h ic s a n d ch a ra ct er is ti cs o f ch il d re n w it h a cr o d er m a ti ti s co n ti n u a o f H a ll o p ea u .

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.

Australasian Journal of DermatologyVolume 62, Issue 2 p. e329-e331 Letter to the Editors The effect of topical sirolimus on superficial Kaposiform haemangioendothelioma Yuan-Xiang Liu, Yuan-Xiang Liu orcid.org/0000-0001-9622-7899 Department of Dermatology, Beijing Children’s Hospital, Capital Medical University, National Center for Children's Health, Beijing, ChinaSearch for more papers by this authorJian Zhang, Jian Zhang Department of Pharmacy, Beijing Children’s Hospital, Capital Medical University, National Center for Children's Health, Beijing, ChinaSearch for more papers by this authorXiao-Lu Nie, Xiao-Lu Nie Center of Clinical Epidemiology＆Evidence-based Medicine, Beijing Children’s Hospital, Capital Medical University, National Center for Children's Health, Beijing, ChinaSearch for more papers by this authorYun-Liu Chen, Yun-Liu Chen orcid.org/0000-0003-2259-6442 Department of Dermatology, Beijing Children’s Hospital, Capital Medical University, National Center for Children's Health, Beijing, ChinaSearch for more papers by this authorZhao-Yang Wang, Zhao-Yang Wang Department of Dermatology, Beijing Children’s Hospital, Capital Medical University, National Center for Children's Health, Beijing, ChinaSearch for more papers by this authorZi-Gang Xu, Corresponding Author Zi-Gang Xu zigangxu@yahoo.com orcid.org/0000-0001-8645-1854 Department of Dermatology, Beijing Children’s Hospital, Capital Medical University, National Center for Children's Health, Beijing, ChinaSearch for more papers by this author Yuan-Xiang Liu, Yuan-Xiang Liu orcid.org/0000-0001-9622-7899 Department of Dermatology, Beijing Children’s Hospital, Capital Medical University, National Center for Children's Health, Beijing, ChinaSearch for more papers by this authorJian Zhang, Jian Zhang Department of Pharmacy, Beijing Children’s Hospital, Capital Medical University, National Center for Children's Health, Beijing, ChinaSearch for more papers by this authorXiao-Lu Nie, Xiao-Lu Nie Center of Clinical Epidemiology＆Evidence-based Medicine, Beijing Children’s Hospital, Capital Medical University, National Center for Children's Health, Beijing, ChinaSearch for more papers by this authorYun-Liu Chen, Yun-Liu Chen orcid.org/0000-0003-2259-6442 Department of Dermatology, Beijing Children’s Hospital, Capital Medical University, National Center for Children's Health, Beijing, ChinaSearch for more papers by this authorZhao-Yang Wang, Zhao-Yang Wang Department of Dermatology, Beijing Children’s Hospital, Capital Medical University, National Center for Children's Health, Beijing, ChinaSearch for more papers by this authorZi-Gang Xu, Corresponding Author Zi-Gang Xu zigangxu@yahoo.com orcid.org/0000-0001-8645-1854 Department of Dermatology, Beijing Children’s Hospital, Capital Medical University, National Center for Children's Health, Beijing, ChinaSearch for more papers by this author First published: 20 November 2020 https://doi.org/10.1111/ajd.13499 Conflict of Interest: none. This study was approved by the institutional review board of the Beijing Children’s Hospital, Capital Medical University. Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat No abstract is available for this article. Volume62, Issue2May 2021Pages e329-e331 RelatedInformation