Rash Research Articles

Feasibility of continuous glucose monitoring in patients with type 1 diabetes at two district hospitals in Neno, Malawi: a randomised controlled trial

ObjectivesTo assess the feasibility and change in clinical outcomes associated with continuous glucose monitoring (CGM) use among a rural population in Malawi living with type 1 diabetes.DesignA 2:1 open randomised...

Disseminated Talaromyces marneffei infection initially presenting as cutaneous and subcutaneous lesion in an HIV-Negative renal transplant recipient: a case report and literature review

BackgroundThe incidence of Talaromyces marneffei (T. marneffei) infection has increased in recent years with the development of organ transplantation and the widespread use of immunosuppressive agents. However, the lack of clinical suspicion leading to delay or misdiagnosis is an important reason for the high mortality rate in non-human immunodeficiency virus (HIV) and non-endemic population. Herein, we report a case of disseminated T. marneffei infection in a non-HIV and non-endemic recipient after renal transplant, who initially presented with skin rashes and subcutaneous nodules and developed gastrointestinal bleeding.Case presentationWe describe a 54-year-old renal transplantation recipient presented with scattered rashes, subcutaneous nodules and ulcerations on the head, face, abdomen, and right upper limb. The HIV antibody test was negative. The patient had no obvious symptoms such as fever, cough, etc. Histopathological result of the skin lesion sites showed chronic suppurative inflammation with a large number of fungal spores. Subsequent fungal culture suggested T. marneffei infection. Amphotericin B deoxycholate was given for antifungal treatment, and there was no deterioration in the parameters of liver and kidney function. Unfortunately, the patient was soon diagnosed with gastrointestinal bleeding, gastrointestinal perforation and acute peritonitis. Then he rapidly developed multiple organ dysfunction syndrome and abandoned treatment.ConclusionsThe risk of fatal gastrointestinal bleeding can be significantly increased in kidney transplant patients with T. marneffei infection because of the long-term side effects of post-transplant medications. Strengthening clinical awareness and using mNGS or mass spectrometry technologies to improve the detection rate and early diagnosis of T. marneffei are crucial for clinical treatment in non-HIV and non-endemic population.

Adult-onset Still’s disease: analysis of a monocentric cohort of patients

Objective Adult-onset Still’s disease (AOSD) is a multigenic autoinflammatory disease with a severe systemic involvement. Because of the rarity of the disease, most published cohorts are multicentric. The aim of this report is to describe a monocentric cohort of AOSD patients, reporting clinical features and response to therapy in a long follow-up. Method Thirty-eight patients, attending the Clinical Immunology Unit and fulfilling Yamaguchi, Fautrel, or Daghor-Abbaci classification criteria for AOSD, were recruited for this study. In all patients, clinical and serological data were collected at diagnosis and every 6 months thereafter. The Pouchot score was calculated at every visit. Results Fever, arthromyalgia, and skin rash were the most frequent manifestations, followed by lymphadenopathy, sore throat, arthritis, splenomegaly, hepatic involvement, pleuropericarditis, and weight loss. As far as the disease course is concerned, 25% presented a monocyclic and 35% a polycyclic pattern, and 40% developed chronic articular involvement. Severe complications were observed at disease onset in 21% of the patients. All of the patients were treated with steroids; 74% also received conventional synthetic disease-modifying anti-rheumatic drugs (methotrexate in most cases) and 71% biological disease-modifying anti-rheumatic drugs (interleukin-1 inhibitors in most cases). Therapeutic switching for lack/loss of efficacy or adverse drug reactions was necessary in 66%. Conclusion The analysis of this cohort confirms that AOSD is a complex, severe, and heterogeneous disease. However, despite long-term treatment and comorbidities, therapies are effective and well tolerated. The therapeutic armamentarium now available allows long-lasting remission with low immunosuppression to be achieved in most patients.

Amoxicillin, gemifloxacin and rabeprazole, as first-line Helicobacter pylori therapy in clinical practice: A pilot study.

Success in eradication of H. pylori is decreasing due to increasing resistant strains. In particular, side-effects due to 4-agent treatment multiple drug use are observed and treatment compliance decreases. The aim of this study was to evaluate the efficacy, reliability, and side-effect profile of the combination of amoxicillin and rabeprazole with gemifloxacin, which is a new generation quinolone, in the treatment of H. pylori infection. This study was conducted on 71 naive patients who received H. pylori eradication. All the patients were administered treatment of Amoxicillin (1000 mg twice a day) + Gemifloxacin (320 mg once a day) + rabeprazole (20 mg twice a day) for 7 days. Drug compliance and treatment tolerance were evaluated after finishing the treatment. At 1 month after the end of the treatment, H. pylori eradication was evaluated in all the patients by examining H. pylori antigen in the feces. In the evaluation after treatment, H. pylori eradication was obtained in 63 (88.7%) patients and eradication was not obtained in 8 (11.3%) patients. The treatment was not completed by 2 patients because of side-effects and noncompliance, so after exclusion of these 2 patients, successful H. pylori eradication was obtained in 63 (91.3%) of 69 patients who completed the treatment. Side-effects were seen in a total of 9 (12.7%) patients. Diarrhea, bloating, abdominal pain, and nausea-vomiting were seen in some patients, but no reflux, constipation, skin rash, listlessness-fatigue, headache, dizziness, palpitations, dry mouth, or weight loss was seen in any patient. In regions with high resistance to clarithromycin and metronidazole in particular, the combination of gemifloxacin with amoxicillin and rabeprazole can be considered for use in first-stage treatment as both the efficacy and tolerability are high.

Clinicopathologic conference: Bloodstream infection in an allogeneic hamatopoietic cell transplant: Thinking beyond the usual.

This case involves a 53-year-old female with concurrent acute myeloid leukemia (AML) and multiple myeloma. She underwent cytarabine and daunorubicin (7+3) induction chemotherapy followed by cytarabine (HiDAC) consolidation, with an early AML relapse requiring azacitidine and venetoclax therapy. She achieved complete remission and incomplete count recovery. Following fludarabine, melphalan, and thymoglobulin induction chemotherapy, she underwent an allogeneic stem cell transplant with failure to engraft, requiring autologous stem cell rescue, buffy coat, and granulocyte transfusions, eventually presenting with a diffuse skin rash consistent with Steven-Johnson syndrome and toxic epidermal necrolysis, persistent neutropenic fevers and positive blood cultures.

Risk factors and early preventive measures for long COVID in non-hospitalized patients: analysis of a large cohort in the United Arab Emirates

ObjectivesLong COVID is characterized by persistent symptoms lasting for 4 weeks or more following the acute infection with SARS-CoV-2. Risk factors for long COVID and the impact of pre-COVID vaccination and treatment during acute COVID-19 remain uncertain. This study aimed to investigate patient-specific factors associated with long COVID in a large cohort of non-hospitalized adult patients with mild to moderate COVID-19 in Dubai. Study designCohort study. MethodsThe study included 28,375 non-hospitalized adult patients diagnosed with mild to moderate COVID-19 between January 1, 2021, and September 31, 2022, in Dubai, who were followed up for 90 days. The presence of long COVID symptoms was documented by physicians during patient visits to the family medicine department. Furthermore, long COVID-related risk factors were collected and analyzed, including patient demographics, comorbidities, pre-COVID vaccination status, and the COVID-related treatments received during the acute phase of the illness. Cox proportional hazard models were applied for the statistical analysis. ResultsAmong the cohort, 2.8% of patients experienced long COVID symptoms during the 90-day follow-up. Patients with long COVID tended to be younger, female, and of Caucasian race. Common symptoms included fatigue, muscle pain, respiratory symptoms, abdominal and neurological symptoms, allergic reactions, skin rashes, and hair loss. Risk factors for long COVID were identified as diabetes mellitus, asthma, and Vitamin D deficiency. Females and Caucasians had a higher risk of long COVID during the pre-Omicron period compared to the Omicron period. Pre-COVID vaccination was associated with a reduced risk of long COVID in all patient subgroups. Treatment with favipiravir or sotrovimab during the acute phase of COVID-19 was linked to a decreased risk of long COVID, although favipiravir showed limited effectiveness in the high-risk group. ConclusionThis study contributes to the existing knowledge by identifying risk factors for long COVID among non-hospitalized patients and emphasizing the potential benefits of pre-COVID vaccination and timely treatment.

Impact of Hazardous Chemical Compounds on the Reproductive System Reported in Sanitary Products

Abstract: A particular group of routine essentials, feminine hygiene items, might expose women to plasticizers and antimicrobial agents. The entire world produces and uses a vast amount of chemicals, some of which may be harmful to reproductive health. The majority of chemicals and work environments have not been investigated for their potential to harm reproductive systems up to this point. The Republic of Korea and other recently industrialized nations have accumulated chemicals and other toxicants quickly, endangering well-being, especially their reproductive systems. (UTC) Women frequently utilize feminine hygiene products (FHPs) as personal care products. ( 2 )According to a broad review, sanitary pads have an impact on endometriosis, vaginal microflora, and vulvar or vaginal skin. Female health has also been found to be impacted by social contexts, such as the use of hygiene products or puberty education. (temp) Chemicals that are dangerous now or in the future are included in the category of sanitary products. Infertility, ovarian cancer, skin rashes, and skin darkening are just a few of the problems that can arise from using sanitary products that contain hazardous chemicals and harm the reproductive system. menstrual cycle imbalance. Sanitary products contain a variety of substances, including VOC, furans, phthalates, bisphenols, and dioxin. Jppd Diapers and sanitary pads are composed of synthetic plastic materials, which may leak when being used. In the present study, the quantities of phthalates (DBP, DEHP, DEP, and BBP) and volatile organic compounds (VOCs) (methylene chloride, toluene, and xylene) in sanitary pads and diapers were measured.

Management and outcome of oncological patients under immune checkpoint inhibitors presenting at the emergency department

IntroductionWith the rising use of immune checkpoint inhibitors (ICIs) in oncology, emergency physicians are increasingly confronted with their immune-related adverse events (irAEs). We described the types of irAEs presenting to...

The Use of Quantum Stem Cell Therapy for Autoimmune Diseases Treatment

This qualitative descriptive study explores the effectiveness of quantum stem cell therapy in addressing autoimmune diseases, with a specific focus on immune modulation and tissue regeneration. Conducted at the Vinski Regenerative Center, the study involved two female patients, aged 53 and 59, who underwent repeated stem cell injections over a specified period. Patient data, encompassing demographic information and disease characteristics, were collected through comparative literature reviews and laboratory analyses. Preliminary findings reveal promising therapeutic outcomes, manifesting as improvements in symptoms such as weakness, joint pain, skin rashes, and fever. Stem cell therapy exhibited potential for immune suppression and tissue repair, thereby offering a novel avenue for managing autoimmune diseases. Further research is imperative to refine treatment protocols and elucidate underlying mechanisms, ultimately enhancing patient care and outcomes in autoimmune disease management.

Skin ‘Bleaching’ Practices and Associated Adverse Health Effects in Zimbabwe: A Canonical Correlational Study of Harare

The study aimed to identify the ingredients, processes and practitioners involved in the skin lightening practices in Zimbabwe and to identify the associated, adverse health effects as well as to establish the user demographics. A technical field survey was carried out in Harare, Zimbabwe from October 2022 to March 2023. Out of a total of 450 potential participants, 382 individuals in 3 broad groups were successfully interviewed; these included 150 skin lightening practitioners and traders, 118 skin lightening products users and 114 individuals who have never practiced skin bleaching. A semi structured technical questionnaire was used for each group of participants and the collected data were analysed qualitatively and quantitatively. The study observed that both sexes are involved in skin lightening in Zimbabwe, the majority (52%) being women aged between 31-45 years. The established industry, is divers, including registered healthcare providers, commercial trade, informal markets and others. The majority of practitioners (65%) have less than 5 practising years. There are over 30 different skin lightening products available either as prescription medications, OTC treatments, commercial products, network marketing products as well as illegal bootleg concoctions. The products extends from creams, lotions and serums, to oral and injectable dosage forms. The most prevalent products being corticosteroid creams. 75% of users reported both reversible and non-reversible adverse health side effects and outcomes including skin irritations, inflammation, rashes, erythema, oedemas, ochronosis, dermal atrophy, photophobia, insomnia and unexplained weight gain. The most prevalent side effect being pruritus and irritations. It was observed that there is no formal registration, regulation or legislation covering the products, processes and practice of skin lightening in Zimbabwe.

Monogenic familial autoinflammatory Вehçet-like syndrome/ haploinsufficiency А20 syndrome is a new form of autoinflammatory pathology. Literature review and description of cases

Monogenic familial autoinflammatory Behçet-like syndrome/haploinsufficiency A20 syndrome is a hereditary autoinflammatory disease from the group of ubiquitinopathies which are caused by a mutation of the TNFAIP3 gene encoding the A20 protein with an autosomal dominant inheritance mechanism and clinical picture similar to Behçet’s disease. Pathogenesis is based on a 50% function decreasing of the nuclear factor inhibitor protein NFkB, what leads to overexpression of proinflammatory cytokines. The disease onset is usually in childhood. Clinical features are presented with recurrent aphthous stomatitis and genital aphthae in most patients and also inflammatory bowel damage is noted. Eye damage is noted rarely than in sporadic Behçet’s disease. In addition, the clinical picture may be presented with arthritis, skin rashes, lesions of the cardiovascular system (pericarditis), fever. Increasing of acute-phase markers is noticed, there is a high frequency of autoantibodies detection in contrast with “classic” autoinflammattory diseases. It can be combined with other autoimmune diseases (systemic lupus erythematosus (SLE), autoimmune thyroiditis, hepatitis, etc.). The description of two patients and comparison with another patient from Russia who was described earlier are presented. All patients had aphthous stomatitis and genital aphthaes, intestinal inflammation symptoms, which was dominanting in one of the patients. Another patient had severe polyarthritis in combination with immunological manifestations which were typical for SLE. The first patient had a good clinical response with the tumor necrosis factor inhibitor adalimumab, the second patient – the anti-B cell drug rituximab.

Cutaneous manifestations of adult-onset Still's disease: A comparative study between young-onset and elderly-onset groups.

We investigated the detailed characteristics and proportions of typical and atypical rashes in 28 patients with adult-onset Still's disease (AOSD) by retrospectively examining the clinical symptoms and pathological features. The patients consisted of six males and 22 females aged between 23 and 85 years. The skin rashes observed in the study population were as follows: (i) typical rash in six cases, (ii) atypical rash in 19, and (iii) both typical and atypical rash in three cases (in all three cases, typical rash preceded atypical rash). Furthermore, we classified atypical rashes into persistent pruritic papules and plaques (PPPP) and others. In 22 cases of atypical rashes, 10 cases had PPPP. In a comparison between young-onset (<65 years) (n = 16) and elderly-onset (≥65 years) (n = 12) AOSD cases, typical rashes were observed more frequently in the young-onset cases. Regarding atypical rashes, PPPP was significantly more common in the elderly-onset cases.

Autoimmune Encephalitis following Checkpoint Inhibitor Therapy in a Patient with Metastatic Melanoma in Complete Remission

The use of immune checkpoint inhibitors (ICIs) in cancer is increasing. Their side effects are mainly due to the triggering of autoimmunity, which are mild or moderate and include skin rash, colitis, hepatitis, endocrine disorders, myositis, interstitial lung disorder, etc., in most cases during the course of therapy. Autoimmune encephalitis (AE) is rare in cancer patients treated with ICIs. Fifty patients with ICI-related encephalitis were identified in a recent review. Herein, we report a case of pembrolizumab associated with AE with a favorable short-term prognosis. A 68-year-old man with malignant metastatic melanoma achieved complete remission after pembrolizumab treatment. However, 10 months after pembrolizumab cessation due to grade 3 diarrhea, he developed confusion, an altered mental status, progressive memory loss, and gait disturbance. He was admitted to the neurologic department, and a comprehensive neurological workup, brain magnetic resonance imaging, cerebral fluid analysis, EEG, and blood test allowed the diagnosis of autoimmune encephalitis. The patient was treated with plasmapheresis, a high dose of intravenous steroids, and intravenous immunoglobulins. The patient improved, and he is now well with a performance status of 1. This case is interesting since the AE developed approximately 10 months after the cessation of immunotherapy, the underlying cancer was in complete remission, and the AE showed a good response after the treatment was performed.

Efficacy and safety of EGFR-TKI combined with WBRT vs. WBRT alone in the treatment of brain metastases from NSCLC: a systematic review and meta-analysis

BackgroundThe efficacy and safety of combining epidermal growth factor receptor tyrosine kinase inhibitors (EGFR-TKIs) with whole-brain radiotherapy (WBRT) for treating brain metastases in non-small cell lung cancer patients remains to be determined.MethodsA systematic search was conducted using databases including PubMed, Embase, Web of Science, Cochrane, Wanfang, and China National Knowledge Infrastructure (CNKI), aiming to identify relevant clinical studies on the treatment of brain metastases originating from non-small cell lung cancer through the combination of EGFR-TKI and WBRT. Statistical analysis was performed utilizing Stata 17.0 software, covering clinical studies published until March 1, 2023.ResultsThis analysis incorporated 23 randomized controlled trials (RCTs), involving a total of 2,025 patients. Of these, 1,011 were allocated to the group receiving both EGFR-TKI and WBRT, while 1,014 were assigned to the WBRT alone group. The findings reveal that the combination of EGFR-TKI and WBRT significantly improves the intracranial objective remission rate (RR = 1.57, 95% CI: 1.42–1.74, p &amp;lt; 0.001), increases the intracranial disease control rate (RR = 1.30, 95% CI: 1.23–1.37, p &amp;lt; 0.001), and enhances the 1-year survival rate (RR = 1.48, 95% CI: 1.26–1.73, p &amp;lt; 0.001). Additionally, this combined treatment was associated with a significant survival advantage (RR = 1.48, 95% CI: 1.26–1.73, p &amp;lt; 0.001) and a reduced incidence of adverse effects (RR = 0.65, 95% CI: 0.51–0.83, p &amp;lt; 0.001), particularly with respect to nausea and vomiting (RR = 0.54, 95% CI: 0.37–0.81, p = 0.002) and myelosuppression (RR = 0.59, 95% CI: 0.40–0.87, p = 0.008). However, no statistically significant differences were observed for diarrhea (RR = 1.15, 95% CI: 0.82–1.62, p = 0.418), and skin rash (RR = 1.35, 95% CI: 0.88–2.07, p = 0.164).ConclusionIn contrast to WBRT alone, the combination of EGFR-TKI and WBRT significantly improves intracranial response, enhancing the objective response rate, disease control rate, and 1-year survival rate in NSCLC patients with brain metastases. Moreover, aside from mild cases of rash and diarrhea, there is no statistically significant increase in the incidence of additional adverse effects. Based on the comprehensive evidence collected, the use of third-generation EGFR-TKI combined with WBRT is recommended as the preferred treatment for NSCLC patients with brain metastases, offering superior management of metastatic brain lesions.Systematic review registrationhttps://www.crd.york.ac.uk/PROSPERO/#, CRD42023415566.

Role of MRI in the diagnosis of Actinomycosis: A Case Report

Actinomycosis is caused by non-spore-forming, anaerobic, gram-positive bacteria called actinomyces. Its wide range of manifestations and non-specific symptoms cause complications by delaying diagnosis. We here present a case of a 34-year-old female with a history of recurrent discharging sinus and skin rashes in the posterior chest wall for 1 year and 6 months. Initially, it was suspected of malignancy with secondary infection. The patient was advised for MRI which shows suspicion of actinomyces. Later on, it was confirmed with a biopsy of the posterior chest wall. Here we present a case to describe the role of MRI in the diagnosis of actinomycosis.

Clinical features of drug reaction with eosinophilia and systemic symptoms: A retrospective study in a reference centre of dermatology in Madagascar

AbstractBackgroundDrug reaction with eosinophilia and systemic symptoms (DRESS) is drug‐induced hypersensitivity reaction that can have fatal complications. Three sets of diagnostic criteria have been proposed, however, consensus is lacking.ObjectivesThis study aimed to describe the causative agents, severity, and the clinical course of patients with DRESS in Antananarivo, Madagascar.MethodsA cross‐sectional study was conducted in patients seen for DRESS, in the department of dermatology at the University Hospital Antananarivo, Madagascar from 2014 to 2022. European Registry of Severe Cutaneous Adverse Reactions (RegiSCAR) criteria was used for the diagnosis. Demographic data, latency periods, clinical and laboratory findings, culprit drugs, and outcomes were assessed.ResultsA total of 24 patients were included. Fifteen patients were female. The three most common culprit drugs were carbamazepine (17 patients), phenobarbital (2 patients), and ampicilline (2 patients). Median onset time (interquartile range) was 20 days (range: 7–62 days). Skin rash was present in all patients, and fever in 18 patients. A total of 12 patients showed two or more internal organs involved. Liver and kidney injuries were the most common visceral manifestation. All patients had eosinophilia. Fifteen patients received systemic corticosteroids. Two mortality cases were reported due to DRESS‐related fulminant liver failure and to nosocomial respiratory infection. Mortality was associated with higher eosinophilia (p = 0.03), higher creatininemia (p = 0.04), and shorter time of latency (p = 0.04).ConclusionsOur study show that DRESS is associated with severe morbidity. Early detection and withdrawal of the culprit dru gis crucial to save life and reduce morbidity.

Analysis and prediction of nutritional outcome of patients with pediatric inflammatory bowel disease from Bahrain

BackgroundInflammatory bowel disease (IBD) is a chronic gastrointestinal disease that causes anorexia, malabsorption, and increased energy requirements. Childhood IBD can significantly impact nutritional status and future health.ObjectiveThis study aimed to analyze the nutritional status of patients with pediatric IBD at presentation and during follow-up and to identify predictors of nutritional outcome.MethodsThis retrospective cohort study reviewed the medical records of children diagnosed with IBD in the Pediatric Department, Salmaniya Medical Complex, Bahrain, 1984 − 2023. Demographic data, clinical characteristics, and anthropometric data were collected. World Health Organization growth standards were used to interpret nutritional status.ResultsOf the 165 patients, 99 (60%) had anthropometric data at presentation, and 130 (78.8%) had follow-up data. Most patients were males (64.6%) and had Crohn’s disease (CD) (56.2%), while 43.8% had ulcerative colitis (UC). The median age at presentation was 10.9 years and the mean follow-up duration was 12.6 years. At presentation, 53.5% of the patients were malnourished, that decreased to 46.9% on follow-up. Thinness was reduced from 27.3% at presentation to 12.1% at follow-up (p = 0.003). There was an increased tendency to normal weight on follow-up (59.6%) compared to time of presentation (46.5%), p = 0.035. Overweightness showed a non-significant increase from 26.3% at presentation to 28.3% at follow-up (p = 0.791). Children with IBD were more likely to become obese when they grow up to adulthood (2.3% versus 20.5%, respectively, p < 0.001). Weight-for-age, and height-for-age at presentation were higher among CD compared to UC, but body mass index (BMI) at follow-up was higher among UC patients (p < 0.05). Thinness at follow up was associated with very early-onset disease (p = 0.02), lower weight and BMI at presentation (p < 0.001 each), younger age at follow-up (p = 0.002), pediatric age group (p = 0.023), lower hematocrit (p = 0.017), and higher C-reactive protein (p = 0.007). Overweight at follow up was associated with increased weight and BMI at presentation (p < 0.001 each), longer disease duration (p = 0.005), older age (p = 0.002), and azathioprine intake (p = 0.026). Considering follow-up duration, univariate analysis exhibited that Bahraini nationality, post-diagnosis disease duration, age at follow-up, occurrence of diarrhea, height, and BMI at presentation were factors that decreased liability to abnormal nutritional status, while CD, history of weight loss, perianal disease, and skin rash, and intake of prednisolone expressed increased liability of abnormal nutritional status (p < 0.05).ConclusionPediatric IBD is associated with a high incidence of malnutrition. Thinness is more prominent at presentation, while overweight is higher on follow-up. Multiple risk factors aggravating abnormal nutritional status were highlighted. Accordingly, nutritional counseling should be prioritized in a multidisciplinary approach.

OA34 Unravelling the role of B cells and interferon dysregulation in juvenile dermatomyositis

Abstract Background/Aims Juvenile dermatomyositis (JDM) is a rare childhood autoimmune disease, characterised by muscle inflammation and skin rash. At present, the exact mechanisms driving JDM pathogenesis remain unclear. Recent investigations have focused on the potential roles of interferon (IFN) dysregulation and B cells. This study aims to explore alterations in B cell populations and their association with disease severity and IFN pathway dysregulation in JDM patients. Methods Transcriptomic, flow cytometry, and clinical data were gathered from a cohort of 61 JDM patients recruited from the UK JDCBS. Clinical demographics and disease variables were extracted from the JDCBS database. Flow cytometry was utilised to examine B cell populations in treatment naïve (‘pre-treatment’ n = 48) and JDM patients on steroids and conventional DMARD treatment, including rituximab (on-treatment n = 24), in parallel with age-matched healthy donors (n = 35). Transcriptomic analysis was performed on the total CD19+ B cells to assess their gene expression profiles in JDM patients. Pathway enrichment analysis was applied to identify the involvement of IFN-related pathways in JDM pathogenesis. Results Flow cytometry analysis revealed a significant expansion of total B cells, (defined as CD19+, p &amp;lt; 0.0001, ANOVA) and immature B cells (defined as CD19+ CD24hi CD38hi, p = 0.0416, Kruskal-Wallis) in pre-treatment JDM patients compared to healthy controls, which returned to levels similar to those in healthy donors in on-treatment JDM patients. An inverse relationship between total B cells and the Childhood Myositis Assessment Scale (CMAS), a measure of muscle strength, was observed in pre-treatment JDM patients, indicating a connection between the expanded B cell population and worse disease. Transcriptomic analysis of B cells illustrated distinctive clustering between pre-treatment JDM patients and healthy controls, based on their type I IFN gene expression profiles. Pathway enrichment analysis confirmed the significant enrichment of IFN-related pathways in B cells of pre-treatment JDM patients. Conclusion This study validates previous observations (Piper et al., 2018) supporting a significant role of B cells in the pathogenesis of JDM and their association with IFN dysregulation. Notably, pre-treatment JDM patients exhibited an expansion of the total B cell population, and of immature B cells, which correlated with disease activity and were restored to normal levels with treatment. Transcriptomic analysis identified distinct IFN-related gene profiles in B cells from pre-treatment JDM patients. These findings offer valuable insights into the complex autoimmune condition of JDM, potentially opening avenues for novel therapeutic strategies. Disclosure A.E.K. Syntakas: None. H.D. Nguyen: None. R. Restuadi: None. B.R. Jebson: None. V. Alexiou: None. L.R. Marshall: None. E. Ralph: None. K. O’Brien: None. D. Cancemi: None. H. Peckham: None. A. Radziszewska: None. N. De Gruijter: None. G.T. Hall: None. S. Castellano: None. C. Ciurtin: None. C.T. Deakin: None. E. Rosser: None. L.R. Wedderburn: None. M. Kartawinata: None. M.G.L. Wilkinson: None.

Expert opinion on the prescription practice of topical calamine lotion for chronic urticaria and other dermatologic conditions in Indian clinical settings

Background: Calamine is suggested as a potentially effective treatment for skin exudation, itching, and rashes. However, there aren't many thorough analyses of the drug prescription patterns for treating urticaria in the Indian literature. This study aimed to gather expert opinions on the prescription practice of topical calamine lotion for chronic urticaria and other dermatologic conditions in Indian clinical settings. Methods: The cross-sectional questionnaire-based survey, comprising 25 questions, gathered data regarding the prescription practice of topical calamine lotion for the management of chronic urticaria and other dermatological conditions. Results: Of the 467 participants, 62% indicated that they would prescribe topical calamine lotion for pruritus management. About 39% of the participants preferred the cream formulation of the calamine lotion for treating diaper dermatitis in 11-25% of patients. About 76% of the respondents stated that they would recommend an oral antihistamine along with topical calamine lotion to treat chronic urticaria. For the treatment of pruritus, 44% of the participants preferred menthol in addition to calamine. Sixty-eight per cent of the respondents stated that approximately 5% of those with chronic urticaria need to be treated with calamine and tetrahydrocurcumin. Most specialists (69.38%) preferred using calamine lotion on 5-10% of patients with itchy skin conditions during pregnancy. Conclusions: Experts recommend the topical calamine lotion for managing various skin conditions including pruritus and chronic urticaria. Specialists recommended it as a safe option for managing itchy skin conditions during pregnancy.

P015 Nodular skin rash associated with relapsing polychondritis, a challenging diagnostic journey to VEXAS syndrome

Abstract Background/Aims VEXAS syndrome, first described in 2020, is a haemato-inflammatory disease caused by a mutation in the gene-UBA1.This syndrome can coexist with or without Myelodysplastic syndrome. What makes this case interesting is its long and challenging diagnostic journey and the involvement of different clinical specialties such as dermatology, ophthalmology, rheumatology, respiratory, haematology, and stroke teams. Ultimately, two years after the initial presentation, this appeared to be the first case of VEXAS with confirmed genetic mutation in the region. VEXAS being a relatively new entity, treating clinician/s were not aware of the possibility initially. Therefore, spreading awareness regarding the clinical picture of VEXAS is prudent. This case report highlights rheumatological and dermatological manifestations of the disease to aid in the timely diagnosis and management of future cases. Methods We report a 74-year-old previously healthy male presented in 2021 with a rash on his legs, arms, and torso since October 2019. It was progressive, painful, palpable, erythematous, non-pruritic, intermittent rash, cleared with hyperpigmentation. He also had oligoarticular arthritis, constitutional symptoms, recurrent episodes of polychondritis, uveitis, bibasal lung fibrosis, and an ESR and CRP above 100. He also had lymphadenopathy above the diaphragm, but the biopsy only showed necrotic material, and the bone marrow biopsy did not show any features of haematological malignancy. After excluding infections, he was started on high-dose oral prednisolone for relapsing polychondritis. The skin and inflammatory markers improved immediately following high doses of steroids. However, whenever steroids were weaned down, even with the help of Methotrexate, his skin was flaring. He also had unprovoked DVT and, while on anticoagulation, developed occipital infarction. He had extensive skin involvement when he had to stop Methotrexate following a chest infection. Results The skin biopsy was inconclusive. There was no significant cytopenia but persistent macrocytosis even after stopping Methotrexate. As a result, he underwent a repeat bone marrow biopsy, which showed Vacuoles and gene UBA1 mutation in 2023. Conclusion The heterogeneity and complexity of the disease are due to multi-organ involvement, including skin, joints, eyes, lungs, blood vessels, and cartilage, simultaneously or cumulatively. Skin involvement in VEXAS can also be vast, ranging from neutrophilic dermatosis, erythema nodosum-like lesions, leukocytoclastic, or urticarial vasculitis. Cutaneous manifestations develop in the majority with VEXAS syndrome and can be the first presenting symptom in this new rare syndrome. Disclosure M. Gunawardena: None. L. Yalakki: None. R. Moitra: None. E.L. Williams: None. H. Wong: None. D. D'Cruz: None.