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Inborn errors of immunity lead to autoimmunity, inflammation, allergy, infection, and/or malignancy. Disease-causing JAK1 gain-of-function (GoF) mutations are considered exceedingly rare and have been identified in only four families. Here, we use forward and reverse genetics to identify 59 individuals harboring one of four heterozygous JAK1 variants. In vitro and ex vivo analysis of these variants revealed hyperactive baseline and cytokine-induced STAT phosphorylation and interferon-stimulated gene (ISG) levels compared with wild-type JAK1. A systematic review of electronic health records from the BioME Biobank revealed increased likelihood of clinical presentation with autoimmunity, atopy, colitis, and/or dermatitis in JAK1 variant-positive individuals. Finally, treatment of one affected patient with severe atopic dermatitis using the JAK1/JAK2-selective inhibitor, baricitinib, resulted in clinically significant improvement. These findings suggest that individually rare JAK1 GoF variants may underlie an emerging syndrome with more common presentations of autoimmune and inflammatory disease (JAACD syndrome). More broadly, individuals who present with such conditions may benefit from genetic testing for the presence of JAK1 GoF variants., (© 2024 Bogunovic et al.)

Although generally well tolerated compared with chemotherapy, molecular targeted therapy used in metastatic melanoma may be associated with life-threatening toxicity. We report the case of a patient with metastatic melanoma treated by dabrafenib plus trametinib who developed intracranial hemorrhage. Physicians should be aware of this rare but life-threatening adverse event of B-rapidly accelerated fibrosarcoma (BRAF) and mitogen-activated protein kinase kinase (MEK) inhibitors. However, they should be also careful about the bleeding origin, which can prove to be a new onset of melanoma metastasis or anticoagulation overdose, or even an uncontrolled arterial hypertension., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

The PIK3CA-related overgrowth spectrum (PROS) encompasses various conditions caused by mosaic activating PIK3CA variants. PIK3CA somatic variants are also involved in various cancer types. Some generalized overgrowth syndromes are associated with an increased risk of Wilms tumor (WT). In PROS, abdominal ultrasound surveillance has been advocated to detect WT. We aimed to determine the risk of embryonic and other types of tumors in patients with PROS in order to evaluate surveillance relevance. We searched the clinical charts from 267 PROS patients for the diagnosis of cancer, and reviewed the medical literature for the risk of cancer. In our cohort, six patients developed a cancer (2.2%), and Kaplan Meier analyses estimated cumulative probabilities of cancer occurrence at 45 years of age was 5.6% (95% CI = 1.35%-21.8%). The presence of the PIK3CA variant was only confirmed in two out of four tumor samples. In the literature and our cohort, six cases of Wilms tumor/nephrogenic rests (0.12%) and four cases of other cancers have been reported out of 483 proven PIK3CA patients, in particular the p.(His1047Leu/Arg) variant. The risk of WT in PROS being lower than 5%, this is insufficient evidence to recommend routine abdominal imaging. Long-term follow-up studies are needed to evaluate the risk of other cancer types, as well as the relationship with the extent of tissue mosaicism and the presence or not of the variant in the tumor samples., (© 2023 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Purpose: There are few data on the practical use of dupilumab by the patients and on the patients' experience with this treatment., Objective: The objective of our study was to describe the experience and perception of dupilumab treatment in patients with atopic dermatitis (AD)., Patients and Methods: We conducted a multicenter retrospective observational study including adult patients with moderate to severe AD treated with dupilumab between January 2017 and December 2021. Clinical characteristics were collected and a questionnaire was sent to all patients. It consisted of different parts including the injection method and different numeric rating scales (NRS) evaluating the patient's satisfaction and the constraints related to the treatment., Results: Eighty-two patients were included and the information was available for 77 patients who responded to the questionnaire. Injection of dupilumab was performed by a nurse in 47% (n=36) of patients and 43% (n=33) were autonomous. Injections were performed by a family member for 7 patients or by the general practitioner (1 patient). A wearing-off of the beneficial effect of dupilumab was reported by 47% of patients leading to shorten the dosing interval. In contrast, dose spacing was reported by 9 patients (11%). After a mean follow-up time of 29.7 ± 10.7 months (median: 27 months), drug survival was 72%. From the patients' perspective, the mean patient's satisfaction NRS score was 7.5 ± 1.8, and the constraints related to the treatment were scored at 3.1 ± 2.1 on NRS., Conclusion: Although AD treatments may contribute to the burden of the disease, dupilumab was associated with a lower burden score, likely reflecting both treatment efficacy and easy of use and patient satisfaction., Competing Interests: The authors report no conflicts of interest in this work., (© 2023 Antoine et al.)

Background: Infantile hemangiomas (IHs) can be part of PHACE (posterior fossa anomalies, hemangioma, arterial anomalies, cardiac anomalies, eye anomalies) syndrome when they are segmental, extensive, and located on the face or neck. The initial assessment is codified and well known, but there are no recommendations for the follow-up of these patients. The aim of this study was to assess the long-term prevalence of different associated abnormalities., Methods: Patients with a history of large segmental IHs of the face or neck. diagnosed between 2011 and 2016 were included in the study. Each patient underwent an ophthalmological, dental, ENT (ear, nose, and throat), dermatological, neuro-pediatric, and radiological assessment at inclusion. Eight patients including five with PHACE syndrome were prospectively evaluated., Results: After a mean follow-up of 8.5 years, three patients presented with an angiomatous aspect of the oral mucosa, two with hearing loss, and two with otoscopic abnormalities. No patients developed ophthalmological abnormalities. The neurological examination was altered in three cases. Brain magnetic resonance imaging follow-up was unchanged in three out four patients and revealed atrophy of the cerebellar vermis in 1 patient. Neurodevelopmental disorders were found in five of the patients and learning difficulties were observed in five patients. The S1 location appears to be associated with a higher risk of neurodevelopmental disorders and cerebellar malformations, while the S3 location was associated with more progressive complications, including neurovascular, cardiovascular, and ENT abnormalities., Conclusion: Our study reported late complications in patients with a large segmental IH of the face or neck, whether associated with PHACE syndrome or not, and we proposed an algorithm to optimize the long-term follow-up., Competing Interests: Declaration of Competing Interest None declared., (Copyright © 2023 French Society of Pediatrics. Published by Elsevier Masson SAS. All rights reserved.)

Background: The long-term effectiveness of immune checkpoint inhibitor (ICI) rechallenge for progressive or recurrent advanced melanoma following previous disease control induced by ICI has not been thoroughly described in the literature., Patients and Methods: In this retrospective multicenter national real-life study, we enrolled patients who had been rechallenged with an ICI after achieving disease control with a first course of ICI, which was subsequently interrupted. The primary objective was to evaluate tumor response, while the secondary objectives included assessing the safety profile, identifying factors associated with tumor response, and evaluating survival outcomes., Results: A total of 85 patients from 12 centers were included in the study. These patients had advanced (unresectable stage III or stage IV) melanoma that had been previously treated and controlled with a first course of ICI before undergoing rechallenge with ICI. The rechallenge treatments consisted of pembrolizumab ( n = 44, 52%), nivolumab ( n = 35, 41%), ipilimumab ( n = 2, 2%), or ipilimumab plus nivolumab ( n = 4, 5%). The best overall response rate was 54%. The best response was a complete response in 30 patients (35%), a partial response in 16 patients (19%), stable disease in 18 patients (21%) and progressive disease in 21 patients (25%). Twenty-eight adverse events (AEs) were reported in 23 patients (27%), including 18 grade 1-2 AEs in 14 patients (16%) and 10 grade 3-4 AEs in nine patients (11%). The median progression-free survival (PFS) was 21 months, and the median overall survival (OS) was not reached at the time of analysis. Patients who received another systemic treatment (chemotherapy, targeted therapy or clinical trial) between the two courses of ICI had a lower response to rechallenge ( p = 0.035) and shorter PFS ( p = 0.016)., Conclusion: Rechallenging advanced melanoma patients with ICI after previous disease control induced by these inhibitors resulted in high response rates (54%) and disease control (75%). Therefore, ICI rechallenge should be considered as a relevant therapeutic option.

Background: Dupilumab is the first biotherapy available for the treatment of moderate-to-severe childhood atopic dermatitis (AD)., Objective: The aim of this study was to evaluate the effectiveness and safety of dupilumab in daily practice., Methods: Patients aged 6-11, who had received a first dose of dupilumab, were included in this multicentre retrospective cohort study. The primary endpoint was change in SCORAD after 3 months of treatment. Secondary endpoints were change in IGA score at 3 months, proportion of patients with SCORAD50 and SCORAD75, description of adverse events and proportion of children in our cohort who would be excluded from pivotal phase 3 clinical trial., Results: Eighty patients were included. After 3 months of treatment, there was a significant decrease in SCORAD (mean: 21.8 ± 13.8 vs 53.9 ± 18.5; P < 0.0001) and IGA (1.3 ± 0.8 vs 3.5 ± 0.7; P < 0.0001). Conjunctivitis was observed in 11.3% (n = 9/80); three patients experienced dupilumab facial redness (DFR); 17.5% (n = 14/80) reported injection site reactions; 6.3% (n = 5/80) discontinued treatment. 61.2% (n = 49/80) children were ineligible in the phase 3 trial., Limitations: There is no control group. Because it was a real life study based on information from patient medical records in a French multicentre cohort, we cannot rule out the presence of reporting bias generated by the use of patient reported characteristics and missing information., Conclusion: These real-life data confirm the efficacy and safety of dupilumab in children with moderate to severe AD extended to dyshidrosis and atopic prurigo, but it also revealed a lower frequency of DFR and conjunctivitis. However, administration in injectable form may be a barrier in this age group., (© 2022 European Academy of Dermatology and Venereology.)

Competing Interests: Conflicts of interest None disclosed.

Introduction: Biological therapies are valuable treatments for severe psoriasis. Children aged under 12 years are underrepresented in therapeutic trials for these drugs. The objective of the 'BiPe Jr' cohort study was to evaluate the drug survival, effectiveness, tolerance and switching patterns of biological therapies in children under 12 years of age with psoriasis., Methods: We conducted a multicentre retrospective study of children with psoriasis who received at least one injection of a biological agent, even off-licence, before the age of 12 years in France and Italy, collecting the data between April and August 2021. The data collected were from March 2012 up to August 2021., Results: In total, 82 children (mean age: 9.1 years; females: 61.0%) received 106 treatments. The drugs administered were adalimumab (n = 49), etanercept (n = 37), ustekinumab (n = 15), anakinra (n = 2), infliximab (n = 2) and secukinumab (n = 1). The most common form of psoriasis was plaque psoriasis (62.9%). The Physician Global Assessment and the Psoriasis Area Severity Index (PASI) scores decreased significantly from baseline to 3 months after treatment initiation for the three main biological drugs; PASI went from 14.1 ± 9.4 to 4.1 ± 11.3 for adalimumab (p = 0.001), 14.9 ± 9.3 to 5.1 ± 4.0 for etanercept (p = 0.002) and 11.6 ± 8.3 to 2.6 ± 2.2 for ustekinumab (p = 0.007). A trend towards higher 2-year maintenance rates was observed for ustekinumab and adalimumab, compared with etanercept (p = 0.06). 52 children discontinued their biological therapy, most frequently due to inefficacy (n = 28) and remission (n = 14). Seven serious adverse events (SAEs) were reported, including four severe infections., Discussion: Our analyses of drug survival and treatment patterns, combined with those of previous studies conducted in older children, indicate that there is a trend towards higher 2-year survival rates of ustekinumab and adalimumab. The SAEs identified were rare, but highlight the need for increased vigilance concerning infections. Overall, the biological therapies showed good effectiveness and safety profiles when used in daily practice for the treatment of young children with psoriasis., (© 2022. The Author(s), under exclusive licence to Springer Nature Switzerland AG.)

CD4 T cells play a key role in anticancer immunity. In this study, we investigate the clinical relevance of circulating CD4 T helper type 1 (Th1) response against telomerase (anti-TERT Th1 response) in patients with melanoma. The spontaneous anti-TERT Th1 response was detected in 54.5% (85/156) of patients with melanoma before treatment. The prevalence of this systemic response was inversely related to Breslow thickness >1 mm and American Joint Committee on Cancer stage ≥II (P = 0.001 and 0.032, respectively). In contrast to patients treated with targeted therapies, the anti-TERT Th1 immunity was associated with an objective response after immune checkpoint inhibitors treatment. Hence, 86% (18/21) of responder patients exhibited pre-existing anti-TERT Th1 versus 35% (6/19) in nonresponders (P = 0.001). This response was also associated with increased progression-free survival and overall survival in patients with melanoma treated with immune checkpoint inhibitors (P = 0.0008 and 0.012, respectively). Collectively, the presence of circulating anti-TERT Th1 response is inversely related to melanoma evolution and appears to be a predictive factor of response to immunotherapy. Our results highlight the interest in telomerase-specific CD4 Th1 response as a promising blood-based biomarker of immune checkpoint inhibitors therapy in melanoma., (Copyright © 2021 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare and potentially fatal adverse reaction. It can be difficult to diagnose, even more so among children, because symptoms may mimic other commonly encountered pediatric conditions., Objective: To describe clinical and laboratory features of DRESS syndrome in the pediatric population (age ≤18 years) and establish causative agents and treatment modalities., Methods: This was a multicenter retrospective study of probable and definite DRESS cases (Registry of Sever Cutaneous Adverse Reaction score ≥ 4) in children hospitalized in 15 French university hospitals between 2000 and 2020., Results: We included 49 cases. All children had fever and rash, 69.4% had lymphadenopathy, and 65.3% had facial edema. The most common organ affected was the liver (83.7%). Treatment consisted of topical corticosteroid in only 30.6% and systemic corticosteroid in 55.1%; 12.2% received intravenous immunoglobulin. Among probable and likely culprit drugs, 65% were antibiotics and 27.5% were antiepileptics, median time to DRESS symptom onset after initiation of 15 days (13 days with antibiotics and 21 days with antiepileptics). Twenty-seven children had allergy assessment for causative agents, 65.4% of whom had positive tests., Conclusions: Culprit drugs are frequently antibiotics and antiepileptic drugs, and onset is often less than 2 weeks after treatment starts, especially with antibiotics. Treatment with topical corticosteroids appears to be sufficient in the least severe cases. Treatment by systemic corticosteroid therapy remains the reference treatment in case of severe organ damage., (Copyright © 2021 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.)

Background: Follow-up of juvenile dermatomyositis (JDM) patients has demonstrated the impact of the disease on several organs in the long term., Objective: As there is little information on the long-term outcome of JDM, we aimed to assess long-term outcomes in a series of JDM patients., Methods: After selection of JDM patients, a consultation with a dermatologist and a rheumatologist was held for each patient. Cutaneous, muscle, and disease damage was assessed using different validated scores including the abbreviated Cutaneous Assessment Tool (aCAT), 8-muscle Manual Muscle Testing (MMT8), Childhood Myositis Assessment Scale (CMAS), Myositis Damage Index (MDI), Childhood Health Assessment Questionnaire (CHAQ), and Health Assessment Questionnaire (HAQ). Long-term disease outcomes were recorded including growth and pubertal development, educational and vocational achievement, and development of comorbidities., Results: Seven patients were included in the study. After a mean follow-up of 14.9±8.8 years, the mean aCAT score was 0.57±1.4 and only one patient had a positive aCAT activity score. The mean aCAT damage score was 1.4±1.3 and five (71%) patients had a score of ≥1. Five (71.4%) patients had normal muscle strength with an MMT8 score of >72, and none had severe muscle weakness (MMT8 ≤32, and CMAS<35). The mean total extent of damage according to the MDI was ≥1 in five (71%) patients and mainly involved the skin. Two (29%) patients had mild disability according to the CHAQ/HAQ disability index. In terms of quality of life, no patient had a score of<40 (1 SD below the mean for healthy controls)., Conclusions: Based on validated cutaneous and musculoskeletal scores, our study demonstrated the good functional outcomes of JDM at long-term follow-up., (Copyright © 2021 French Society of Pediatrics. Published by Elsevier Masson SAS. All rights reserved.)

Background: Dissecting cellulitis of the scalp (DCS), also known as Hoffmann disease or perifolliculitis capitis abscedens et suffodiens, is a rare disease characterized by chronic inflammation of the scalp. Treatment is difficult and often disappointing., Objectives: To report our experience of TNF inhibitors in a series of patients with DCS., Materials & Methods: We conducted a monocentric retrospective study of nine patients with DCS treated with TNF blocker after failure of other conventional treatments., Results: After a mean duration of treatment by TNF inhibitors of 17 ± 16 months, four patients (44% versus 0%) had a Physician's Global Assessment score of 0 or 1. We observed a 67% reduction in the number of inflammatory nodules, an 88% reduction in purulent drainage and a 45% improvement in Dermatology Life Quality Index. The mean treatment satisfaction index was 6.6 ± 1.6 out of 10., Conclusion: Our study suggests that TNF inhibitors are effective against disease activity and may improve quality of life in the management of DCS refractory to conventional treatments.

Angiosarcoma (AS) is a rare aggressive sarcoma with differentiation toward blood or lymphatic endothelium. There are few epidemiological data available on AS. To address this limitation, we investigated the epidemiological and clinical features of angiosarcoma diagnosed in a French administrative area (the Doubs department) from 1979 to 2016. A retrospective cohort study was conducted using the Doubs cancer registry database. A total of 45 patients with invasive AS were diagnosed between 1979 and 2016 in the Doubs department. Among the 45 AS, 51% were either cutaneous AS (27%), including head and neck and extremities, or breast AS (24%) as compared to visceral AS (42%). Eleven patients had metastasis at diagnosis (26%). Age-standardized incidence rate was 0.15 per 100,000 persons-years (95%CI, 0.10-0.20) for the entire study period (1979-2016) and 0.26 (95%CI, 0.15-0.42) for the last decade (2007-2016). Crude survival at 1, 3, 5 years after diagnosis was 44%, 21%, and 12%, respectively. Our population-based study provides updated data on the incidence and overall survival of AS in a French population-based cancer registry., Competing Interests: Conflict of interest: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2020.)

In the setting of metastatic uveal melanoma (mUM), prognosis is dismal and treatment options are limited. MEK inhibition using selumetinib has led to promising results with improved progression-free survival. While immune checkpoint inhibitors such as programmed cell death-1 (PD-1) blockade therapy (anti-PD-1) has shown discrete efficacy in mUM, combining MEK inhibitors (MEKi) to anti-PD-1 might be an option as such combinations have shown synergistic efficacy in metastatic cutaneous melanoma. We report here and discuss our experience in three patients who received this combination in the absence of suitable alternative treatment. The efficacy was difficult to assess due to early severe toxicities (pneumonitis and Takotsubo syndrome). This case series highlights the need to evaluate the safety and efficacy of new treatment options such as MEKi and anti-PD-1 for mUM.

Background: Although infliximab (IFX) has been available since 2005, there are very little data on the long-term drug survival of infliximab in real-life., Objective: Our aim was to identify and describe psoriasis patients treated with IFX for longer than 6 years., Methods: Psoriasis patients treated with IFX for longer than 6 years were retrospectively included. Demographic and clinical data were collected in May 2018., Results: Between January 2005 and December 2012, 43 patients were maintained on IFX for 6 years or longer. IFX was introduced as a 4.5 line of systemic therapy. The mean duration of IFX treatment was 8.5 years (6-12). In May 2018, 30 patients (70%) were still maintained on IFX at 4-6 mg/kg every 8-10 weeks with an efficiency of about 100%. IFX was stopped in 13 patients (30%) mainly for loss of efficacy in 6 patients (46%). Three patients developed solid cancer including bladder cancer, lung cancer, and prostate cancer. Limitation . Retrospective study., Conclusion: We report the efficacy and safety of IFX maintained for up to 12 years in psoriasis patients. The long-term use of IFX was associated with a high BMI confirming the critical role of weight-based dosing for this drug., Competing Interests: François Aubin, Emmanuel Mahé, Denis Jullien, and Marie Aleth Richard received honoraria as a consultant and/or investigator and/or speaker for and/or have been reimbursed for conference attendance by Abbvie, Amgen, Boehringer, Celgene, GSK, Janssen-Cilag, Leo Pharma, Lilly, MSD, Nordic, Novartis, Pfizer, MSD, Sanofi, and Sandoz. Axel Villani received honoraria as a consultant for Novartis and Janssen-Cilag., (Copyright © 2020 Clémentine Carlet et al.)

Background: Belimumab is currently approved for the treatment of active systemic lupus erythematosus (SLE). The aim of our study was to evaluate the efficacy of belimumab in the treatment of cutaneous lupus erythematosus (CLE), resistant to conventional therapy., Patients and Methods: Seven patients with resistant and progressive LEC and treated with belimumab were retrospectively analyzed. The efficacy and safety of belimumab were evaluated with the CLASI, RCLASI and DLQI scores, after 6 to 12 months of treatment., Results: Eighty-three percent of patients demonstrated a significant clinical improvement based on the CLASI and RCLASI activity scores, including 1 complete and 4 partial responses, without worsening of CLASI and RCLASI damage scores. Eighty percent of patients also showed an improvement of their quality of life (DLQI). Oral corticosteroids were discontinued in all patients. Tolerance was acceptable with only one serious adverse event (bacteriema)., Conclusion: Our study suggests the clinical efficiency of belimumab in a series of 7 patients presenting a resistant and progressive CLE., (Copyright © 2019 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.)

Introduction: Incontinentia pigmenti (IP) is an X-linked genodermatosis caused by mutation of the NEMO/IKBKG gene. While lethal in male foetuses, heterozygous females survive because of X-inactivation mosaicism. Herein we discuss 9 male patients with IP., Materials and Methods: This is an observational, descriptive, retrospective, multicentre, French study carried out with the help of the SFDP research group. Statistical analysis was performed both on our own patients and on those reported in the literature., Results: Nine boys with no family history of IP but with typical neonatal skin reactions were included. Genetic analysis of blood (n=8) and skin biopsy (n=3) confirmed the diagnosis of IP by identification of common deletion of the IKBKG/NEMO gene (exons 4 to 10) in the state of somatic mosaic in 6 and 2 cases respectively. Where analysed, the karyotype was normal (n=6). Over a median follow-up period of 48 months (3 months to 10 years), 3 patients had neurological abnormalities, 2 had severe ophthalmologic abnormalities, and 1 had dental abnormalities. Extensive skin involvement is a systemic risk factor, unlike cutaneous scarring., Conclusion: IP in boys is often due to a mosaic mutation that should be sought in blood and skin. Long-term neurological and ophthalmological monitoring is essential, especially in cases of extensive skin involvement., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Background: Three biotherapies - etanercept, adalimumab and ustekinumab - are licensed in childhood psoriasis. The few data available on their efficacy and tolerance are mainly derived from industry trials. However, biological drug survival impacts long-term performance in real-life settings., Objective: The objective of this study was to evaluate the survival rates of biological therapies in children with psoriasis in real-life conditions. Secondary objectives were to evaluate the factors associated with the choice of the biological therapy and to report severe adverse events., Materials and Methods: This study was an observational retrospective study. Data were extracted from the clinical records of 134 children. Kaplan-Meier estimates were used to analyse drug survival overall and in subgroups of plaque psoriasis, bio-naïve and non-naïve patients., Results: We analysed 184 treatment courses: 70 with etanercept, 68 with adalimumab and 46 with ustekinumab. Factors associated with the choice of first-line biological agent were age at initiation (younger for adalimumab, P < 0.0001), age at onset of psoriasis (younger for adalimumab and etanercept, P = 0.03) and baseline Psoriasis Assessment Severity Index and Physician global assessment (both higher for adalimumab, P < 0.001). Drug survival rates were higher for ustekinumab than for adalimumab and etanercept (P < 0.0001) for all treatment and all psoriasis types, plaque-type psoriasis (P = 0.0003), patients naïve for biological agents (P = 0.0007) and non-naïve patients (P = 0.007). We reported eight serious adverse events (SAEs): severe infections (n = 3), significant weight gain (n = 2), psoriasis flare (n = 1) and malaise (n = 1). Biological therapy was discontinued in three children (one with psoriasis flare and two with weight gain). Only the two cases of weight gain resulted in an unfavourable outcome., Conclusions: Our real-life comparative study found that ustekinumab had the best drug survival outcome. The profile of SAEs in children was comparable to that in adults. These results will assist dermatologists in the decision-making process when choosing treatment options for children with psoriasis in daily practice., (© 2019 European Academy of Dermatology and Venereology.)

Background: Drug survival in a real-life setting is critical to long-term use of biologics for psoriasis. Objective : We describe our 12-year experience with biologics in psoriasis patients. Patients and Methods: All patients treated with biologics including infliximab, adalimumab (ADA), etanercept (ETA), and ustekinumab (UST) for psoriasis vulgaris between January 2005 and December 2016 were retrospectively analyzed. Results: In total, 545 treatment series were administered to 269 patients, including 211 treatment series with ADA, 135 with ETA, 77 with infliximab, and 122 with UST. ADA and ETA were initiated most often as first-line therapy; 65.3% of treatment sequences were discontinued. UST had the highest drug survival. The major reason for treatment termination was a loss of efficacy (44.9%). Definitive discontinuation increased with the number of biologic therapy sequences. Limitations: Subjects were not randomized to the different treatments. Conclusions: In a long-term real-life setting, drug survival of UST is better than that of TNF-a inhibitors for both biologic-naive and biologic-experienced patients with psoriasis.

The recent advent of immune checkpoint inhibitors (ICI), including anti-programmed cell death 1 protein (anti-PD-1) agents has revolutionized the therapeutic approach of metastatic malignancies. Yet, ICI can disrupt immune tolerance resulting in enhanced immune activation in normal tissues with significant toxicity. A dysregulated activation of T-cells directed to normal tissues stands as the main mechanism of immune-related adverse events (irAE). To date, only two cases of immune-related inflammatory orbitopathy related to anti-PD-1 agents have been reported. This rare immune adverse event usually occurred early after ICI initiation. Here, we report the first case of late inflammatory orbitopathy occurring in a melanoma patient treated with pembrolizumab. Consequently, the occurrence of irAE under ICI should be monitored, even late after treatment instauration.

An accurate experimental determination of electronic structures in semi-conductor nanopowders is a challenging task. We propose here to combine UPS and UV-Vis spectroscopies in order to get the full description of the electronic band alignment of powder samples, TiO2 rutile and anatase. For UPS measurements, two preparation methods, namely the dropping method and electrophoretic deposition, were used to prepare layers of titania powders on a conducting substrate, ITO or Ag. Both methods lead to comparable results, with a quantitative description of the energy levels from the valence band. Combining these results with the UV-Vis spectra of the same powders enables the determination of the absolute position of the valence band maximum and the conduction band minimum. Combined UPS-UV-Vis spectroscopy provides a better insight into the properties of a powdered material which can differ from single crystal model systems. It can also be used to predict the electronic transfer in mixed phase systems during photocatalytic processes.