A 16-year-old patient presented with annular erythema with mild central hypopigmentation. Dermoscopy revealed punctate and hairpin vessels on a yellow-red background. A diagnosis of annular psoriasis was confirmed, with marked improvement after ustekinumab therapy.

INTRODUCTION AND IMPORTANCE:This study aims to evaluate the microsuture technique in treating giant dissecting aneurysms of the middle cerebral artery (MCA) trunk, particularly in cases involving perforators-a key surgical challenge. METHODS:This reconstruction of the MCA trunk was performed in four patients with giant dissecting aneurysms using a microsuture technique. The fragile wall of the dissecting aneurysm was meticulously excised while preserving the perforating arteries originating from the aneurysm body. Subsequently, a bypass was established from the internal maxillary artery (IMA) to the distal MCA (or specify the exact branch if applicable). This surgical approach achieved temporary vascular occlusion while maintaining distal perfusion, thereby preventing ischemia in the territory distal to the aneurysm during its definitive management. OUTCOMES:Among the four patients, three had surgically clipped aneurysms of the MCA trunk, while the remaining patient presented with severe headaches and left lower limb paralysis. All patients underwent microsuture of the MCA trunk and subsequently experienced uneventful postoperative recoveries without neurological deficits. Postoperative angiography demonstrated successful MCA trunk reconstruction with complete dissecting aneurysm resolution in three cases. In the remaining patient, although MCA reconstruction was not radiographically visible, patency of the IMA-to-M2 segment bypass was confirmed. Follow-up imaging revealed: At six-year follow-up, one patient exhibited both a patent IMA-to-M2 bypass and reconstructed M1 trunk on CT angiography; Two patients showed M1 trunk restoration with aneurysm disappearance at three-month follow-up; One patient maintained a patent IMA-to-M2 bypass without visualized MCA trunk. No ischemic events occurred in any patient, presumably due to preserved perforating arteries originating from the reconstructed MCA trunks. CONCLUSION:Some large dissecting aneurysms of the MCA trunk can be effectively treated with the microsuture technique by reconstructing the trunk and preserving its perforators.

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.

HomeStrokeAhead of PrintInsights on Moyamoya Disease: Vascular Inflammation and Nuclear Autoschizis in Cranial Arteries No AccessCase ReportRequest AccessAboutView PDFSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toNo AccessCase ReportRequest AccessInsights on Moyamoya Disease: Vascular Inflammation and Nuclear Autoschizis in Cranial Arteries Xiaoling Ruan, Ting Lei, Xin Xiang, Fangjun Liu and Xiang’en Shi Xiaoling RuanXiaoling Ruan https://orcid.org/0009-0000-7219-384X Department of Neurosurgery, University of Pavia, Italy (X.R.). Search for more papers by this author , Ting LeiTing Lei https://orcid.org/0000-0001-8970-1986 Department of Neurosurgery, Sanbo Brain Hospital (T.L., X.X., F.L.) Department of Neurosurgery, Fuxing Hospital, Capital Medical University, Beijing, China (T.L., X.S.). Search for more papers by this author , Xin XiangXin Xiang https://orcid.org/0009-0000-1897-7539 Department of Neurosurgery, Sanbo Brain Hospital (T.L., X.X., F.L.) Search for more papers by this author , Fangjun LiuFangjun Liu https://orcid.org/0000-0003-2428-6456 Department of Neurosurgery, Sanbo Brain Hospital (T.L., X.X., F.L.) Search for more papers by this author and Xiang’en ShiXiang’en Shi Correspondence to: Xiang’en Shi, MD, PhD, Department of Neurosurgery, Fuxing Hospital, Capital Medical University, No. 20, Fuxingmen Wai St, Xicheng, Beijing 100045, China. Email E-mail Address: [email protected] https://orcid.org/0000-0002-9491-0499 Department of Neurosurgery, Fuxing Hospital, Capital Medical University, Beijing, China (T.L., X.S.). Search for more papers by this author Originally published29 Nov 2023https://doi.org/10.1161/STROKEAHA.123.045064Stroke. 2023;0FootnotesFor Sources of Funding and Disclosures, see page xxx.Correspondence to: Xiang’en Shi, MD, PhD, Department of Neurosurgery, Fuxing Hospital, Capital Medical University, No. 20, Fuxingmen Wai St, Xicheng, Beijing 100045, China. Email shixen@ccmu.edu.cn eLetters(0)eLetters should relate to an article recently published in the journal and are not a forum for providing unpublished data. Comments are reviewed for appropriate use of tone and language. Comments are not peer-reviewed. Acceptable comments are posted to the journal website only. Comments are not published in an issue and are not indexed in PubMed. Comments should be no longer than 500 words and will only be posted online. References are limited to 10. Authors of the article cited in the comment will be invited to reply, as appropriate.Comments and feedback on AHA/ASA Scientific Statements and Guidelines should be directed to the AHA/ASA Manuscript Oversight Committee via its Correspondence page.Sign In to Submit a Response to This Article Previous Back to top Next FiguresReferencesRelatedDetails Advertisement Article InformationMetrics © 2023 American Heart Association, Inc.https://doi.org/10.1161/STROKEAHA.123.045064PMID: 38018829 Originally publishedNovember 29, 2023 KeywordsCTLA4 proteinelectron microscopyhumanimmunohistochemistryinflammationmoyamoya diseasetumor necrosis factor-alphaPDF download Advertisement SubjectsGrowth Factors/CytokinesSmooth Muscle Proliferation and DifferentiationStenosisVascular Disease

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.

Background Adamantinomatous craniopharyngiomas (ACPs) are rare benign epithelial tumours with high recurrence and poor prognosis. Biological differences between recurrent and primary ACPs that may be associated with disease recurrence and treatment have yet to be evaluated at the proteomic level. In this study, we aimed to determine the proteomic profiles of paired recurrent and primary ACP, gain biological insight into ACP recurrence, and identify potential targets for ACP treatment.Method Patients with ACP (n = 15) or Rathke's cleft cyst (RCC; n = 7) who underwent surgery at Sanbo Brain Hospital, Capital Medical University, Beijing, China and received pathological confirmation of ACP or RCC were enrolled in this study. We conducted a proteomic analysis to investigate the characteristics of primary ACP, paired recurrent ACP, and RCC. Western blotting was used to validate our proteomic results and assess the expression of key tumour-associated proteins in recurrent and primary ACPs. Flow cytometry was performed to evaluate the exhaustion of tumour-infiltrating lymphocytes (TILs) in primary and recurrent ACP tissue samples. Immunohistochemical staining for CD3 and PD-L1 was conducted to determine differences in T-cell infiltration and the expression of immunosuppressive molecules between paired primary and recurrent ACP samples.Results The bioinformatics analysis showed that proteins differentially expressed between recurrent and primary ACPs were significantly associated with extracellular matrix organisation and interleukin signalling. Cathepsin K, which was upregulated in recurrent ACP compared with that in primary ACP, may play a role in ACP recurrence. High infiltration of T cells and exhaustion of TILs were revealed by the flow cytometry analysis of ACP.Conclusions This study provides a preliminary description of the proteomic differences between primary ACP, recurrent ACP, and RCC. Our findings serve as a resource for craniopharyngioma researchers and may ultimately expand existing knowledge of recurrent ACP and benefit clinical practice.

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。

ImportanceKeratinopathic ichthyosis (KPI) represents a group of predominantly autosomal dominant genodermatoses resulting from mutations in the KRT1, KRT2, or KRT10 genes. In KPI, the relationship between genotype and phenotype is complex. ObjectiveTo analyze the clinical manifestations and gene mutations in Chinese patients with KPI. MethodsClinical data were collected from 13 children diagnosed with KPI, and peripheral blood DNA samples were extracted from both the patients and their parents Next-generation sequencing was performed using a congenital ichthyosis multi-gene panel, and the selected variants in the patients and their parents were further validated using the Sanger sequencing method. ResultsGenetic analysis identified missense mutations in either KRT1 or KRT10 in ten patients exhibiting varying degrees of severity and distinct features of epidermolytic ichthyosis. A missense hotspot mutation in KRT2 was identified in one patient with superficial epidermolytic ichthyosis. Additionally, two truncation mutations in KRT10 were detected, leading to the development of generalized ichthyosiform erythroderma. Ear malformation and ectropion at birth, scalp involvement, and palmoplantar hyperkeratosis were observed as early signs of ichthyosis with confetti. InterpretationWe analyzed the genotype-phenotype correlations in KPI, revealing that the types and locations of different mutations are associated with distinct phenotypic characteristics. Oral acitretin could be considered a treatment option for severe patients at an appropriate dosage and timing.

Objective:To analyze the incidence and prognosis of epilepsy in frontotemporal lobe glioma.Methods:The clinical data of 208 patients with frontotemporal lobe gliomas in Sanbo Brain Hospital Capital Medical University from 2019 to 2021 were analyzed retrospectively. According to the 2016 World Health Organization (WHO) classification of tumors of the central nervous system, the incidence of epilepsy, Modified Rankin Scale (MRS) score, and Engel Outcome Scale of patients with different grades of tumors were calculated.Results:Among all the patients with frontotemporal lobe gliomas, there was more males than females, and it was more common in the 40 -59 age group. The incidence of epilepsy associated with WHO grade Ⅰand Ⅱ glioma was 100.0% (33/33) and 60.9% (14/23), respectively, while that of WHO grade Ⅳ glioma was 19.0%(19/100). The average follow-up time was (22 ± 9) months. During the follow-up period, the incidence of WHO grade Ⅰ, Ⅱ and Ⅲ glioma-related epilepsy decreased significantly. There was no significant difference in the incidence of glioma-related epilepsy between the total and subtotal resection groups ( P>0.05). There was no statistical correlation between the side of tumor occurrence and the occurrence of epilepsy ( P>0.05), also between the gene phenotype and the occurrence of epilepsy ( P>0.05). There was no significant difference in the Engel Outcome Scale among different grades of gliomas ( P>0.05). The prognosis of patients with Engel Outcome Scale Class 1 was significantly better than that of other grades. Conclusions:The incidence of glioma-related epilepsy is negatively correlated with tumor grade. Age and sex are risk factors for glioma-related epilepsy. The incidence of postoperative epilepsy in patients with low grade glioma is significantly lower than that in patients with high grade glioma, and the prognosis is better. However, there is no significant difference in the Engel Outcome Scale among different grades of gliomas.

Urticarial vasculitis (UV) is a small vessel leucocytoclastic vasculitis, which often needs to be distinguished from urticaria and other dermatoses. Treatment of UV in children is challenging because of the unsatisfying efficacy of antihistamines and the safety concern of long-term systemic corticosteroids or immunosuppressive agents. As a classic biological agent widely used in chronic spontaneous urticaria, omalizumab might also be a potential therapeutic option in the treatment of children with UV. This report presented four children, aged 4-6 years, with glucocorticoid-unresponsive UV successfully treated by omalizumab, thus providing evidence that omalizumab can be used to treat UV with good efficacy and tolerability in the paediatric population. This report presents four children, aged 4-6 years, with glucocorticoid-unresponsive urticarial vasculitis (UV) successfully treated by omalizumab, thus providing evidence that omalizumab is well tolerated and effective in treating UV in the paediatric population.

Sehr geehrte Herausgeber, Ein 9-jähriger Junge stellte sich mit Nagelund Schleimhautläsionen in unserer Klinik vor. Außerdem klagte er über leichte Dysphagie. Vor sieben Jahren begann bei ihm eine Dystrophie der Fingernägel beider Hände, die sich mit der Zeit verschlimmerte (Abbildung 1a). Vor vier Jahren trat eine Leukoplakie an der Zunge und der Mundschleimhaut auf (Abbildung 1b). Bei der Untersuchung wiesen alle zehn Fingernägel eine Dystrophie sowie Hyperpigmentierung auf. Eine netzartige Hyperpigmentierung wurde auch an Hals, Rumpf und Oberschenkel festgestellt (Abbildung 1c). Die Zunge war gerötet, und auf der Zunge und der Mundschleimhaut fanden sich Leukoplakie-Bereiche unterschiedlicher Größe, die die Mundöffnung beeinträchtigten. Vor einem Jahr begann der Patient eine leichte Dysphagie zu entwickeln. Das vollständige Blutbild sowie die Leberund Nierenfunktionstests lagen im normalen Bereich. Die Familienanamnese ergab, dass seine Eltern bei guter Gesundheit waren. Wir entnahmen ein Nagelstanzbiopsat (Abbildung 1d) und führten einen Gentest durch. Die Gewebeprobe zeigte eine Ausdünnung des Nagelbettepithels mit Abflachung der Reteleisten und ein mäßiges interstitielles lymphozytäres Infiltrat in der Dermis ohne lichenoideMerkmale. Das Ergebnis desGentests ergab eine c.1058C > T, p. A353V-Mutation im X-chromosomalen Gen DKC1. Eine Röntgenaufnahme des oberen Verdauungstrakts wies auf eine leichte Stenose der oberen Speiseröhre

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.

HomeStrokeVol. 54, No. 12Unveiling Bacterial Autophagosomes in Human Intracranial Aneurysm Wall Tissue: A Rare Insight No AccessCase ReportRequest AccessFull TextAboutView Full TextView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toNo AccessCase ReportRequest AccessFull TextUnveiling Bacterial Autophagosomes in Human Intracranial Aneurysm Wall Tissue: A Rare Insight Ting Lei, Xiang’en Shi, Xiaoling Ruan, Fangjun Liu and Xin Xiang Ting LeiTing Lei https://orcid.org/0000-0001-8970-1986 Department of Neurosurgery, Fuxing Hospital (T.L., X.S., X.X.), Capital Medical University, Beijing, People’s Republic of China. Department of Neurosurgery, Sanbo Brain Hospital (T.L., X.S., F.L., X.X.), Capital Medical University, Beijing, People’s Republic of China. Search for more papers by this author , Xiang’en ShiXiang’en Shi Correspondence to: Xiang’en Shi, MD, PhD, Department of Neurosurgery, Fuxing Hospital, Capital Medical University, No. 20, Fuxingmen Wai St, Xicheng District, Beijing 100045, People’s Republic of China. Email E-mail Address: [email protected] https://orcid.org/0000-0002-9491-0499 Department of Neurosurgery, Fuxing Hospital (T.L., X.S., X.X.), Capital Medical University, Beijing, People’s Republic of China. Department of Neurosurgery, Sanbo Brain Hospital (T.L., X.S., F.L., X.X.), Capital Medical University, Beijing, People’s Republic of China. Search for more papers by this author , Xiaoling RuanXiaoling Ruan University of Pavia, Milan, Italy (X.R.). Search for more papers by this author , Fangjun LiuFangjun Liu https://orcid.org/0000-0003-2428-6456 Department of Neurosurgery, Sanbo Brain Hospital (T.L., X.S., F.L., X.X.), Capital Medical University, Beijing, People’s Republic of China. Search for more papers by this author and Xin XiangXin Xiang https://orcid.org/0009-0000-1897-7539 Department of Neurosurgery, Fuxing Hospital (T.L., X.S., X.X.), Capital Medical University, Beijing, People’s Republic of China. Department of Neurosurgery, Sanbo Brain Hospital (T.L., X.S., F.L., X.X.), Capital Medical University, Beijing, People’s Republic of China. Search for more papers by this author Originally published12 Oct 2023https://doi.org/10.1161/STROKEAHA.123.044829Stroke. 2023;54:e498–e499"Unveiling Bacterial Autophagosomes in Human Intracranial Aneurysm Wall Tissue: A Rare Insight." Stroke, 54(12), pp. e498–e499FootnotesFor Sources of Funding and Disclosures, see page e499.Correspondence to: Xiang’en Shi, MD, PhD, Department of Neurosurgery, Fuxing Hospital, Capital Medical University, No. 20, Fuxingmen Wai St, Xicheng District, Beijing 100045, People’s Republic of China. Email shixen@ccmu.edu.cnREFERENCES1. Hallikainen J, Pyysalo M, Keränen S, Kellokoski J, Koivisto T, Suominen AL, Pussinen P, Pessi T, Frösen J. Systemic immune response against the oral pathogens Porphyromonas gingivalis and Aggregatibacter actinomycetemcomitans is associated with the formation and rupture of intracranial aneurysms.Eur J Neurol. 2021; 28:3089–3099. doi: 10.1111/ene.14986CrossrefGoogle Scholar eLetters(0)eLetters should relate to an article recently published in the journal and are not a forum for providing unpublished data. Comments are reviewed for appropriate use of tone and language. Comments are not peer-reviewed. Acceptable comments are posted to the journal website only. Comments are not published in an issue and are not indexed in PubMed. Comments should be no longer than 500 words and will only be posted online. References are limited to 10. Authors of the article cited in the comment will be invited to reply, as appropriate.Comments and feedback on AHA/ASA Scientific Statements and Guidelines should be directed to the AHA/ASA Manuscript Oversight Committee via its Correspondence page.Sign In to Submit a Response to This Article Previous Back to top Next FiguresReferencesRelatedDetails December 2023Vol 54, Issue 12 Advertisement Article InformationMetrics © 2023 American Heart Association, Inc.https://doi.org/10.1161/STROKEAHA.123.044829PMID: 37823306 Originally publishedOctober 12, 2023 KeywordsDNADNA probeselectronshumanspolymerase chain reactionPDF download Advertisement SubjectsAneurysm

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.

JDDG: Journal der Deutschen Dermatologischen GesellschaftEarly View CLINICAL LETTER A case of dyskeratosis congenital associated with esophageal stricture in a young child Xin Xiang, Xin Xiang 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, Chaoyang Miao 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, Corresponding Author Zigang Xu [email protected] Department of Dermatology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China Correspondence Zigang Xu, MD, 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 Xin Xiang, Xin Xiang 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, Chaoyang Miao 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, Corresponding Author Zigang Xu [email protected] Department of Dermatology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China Correspondence Zigang Xu, MD, 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: 06 June 2023 https://doi.org/10.1111/ddg.15090 The first two authors contributed equally to this work. AboutPDF 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. Early ViewOnline Version of Record before inclusion in an issue RelatedInformation