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Coronavirus Disease 2019 (COVID-19), caused by SARS-CoV-2, has negatively affected global health. COVID-19 has been associated with a variety of autoimmune and inflammatory disorders, complicating its respiratory manifestations. SARS-CoV-2 triggers inflammatory reactions which may involve multiple organs and systems. The proof for IgA involvement in the immune reactions to coronavirus infection is growing, particularly in the case of IgA immune complex deposition diseases such as IgA vasculitis (IgAV) and IgA nephropathy. This report presents a case of IgAV caused by SARS-CoV-2 in a 53-year-old man. His symptoms included papillomatous, bright red rashes, urticaria throughout the body, aphthous stomatitis, pain in all joints and muscles, weakness, malaise, abdominal pain, face swelling, and arterial hypertension (160/100 mmHg). He received intravenous methylprednisolone (250 mg) and then oral methylprednisolone (16 mg) treatment, which improved his condition. This improvement included the disappearance of abdominal and joint pain and skin rashes. This article also provides an overview of published cases of IgAV after SARS-CoV-2. It may alert rheumatologists and allied specialists of clinical features of IgAV and guide them how to diagnose and treat this disease. [ABSTRACT FROM AUTHOR]

Background and objective: Systemic sclerosis (SSc) is a highly heterogeneous disease whose treatment is based mainly on immunosuppressants, antifibrotics, and vasodilators. Intravenous immunoglobulin (IVIG) have proved effective in other autoimmune diseases. The objective of this study is to evaluate the efficacy and safety of IVIG in SSc. Methods: The systematic review was conducted according to the PRISMA Statement. Medline, Embase and Cochrane Library databases were searched until March 2024. We assessed the quality of included studies using the Cochrane Risk of Bias 2.0 tool (RoB 2) for randomised clinical trials and the Cochrane Risk in non-randomized studies (ROBINS-I) tool for observational studies. Results: From 1242 studies identified, 15 studies were included, of which 14 were observational studies. In total, 361 patients with SSc were included, and 295 received treatment with IVIG. Most of the studies used a dose of 2 g/kg IVIG. Ten studies, including the clinical trial, showed high risk of bias, and five had a critical risk. Skin involvement was assessed using modified Rodnan skin score, in 11 studies and the authors reported cutaneous efficacy in 9 of them. The 6 studies that assessed muscle involvement reported an improvement. Six studies reported data on gastrointestinal efficacy. Other domains such as lung and joint involvement and steroid-sparing effect were evaluated. The most frequent adverse events were mild, including headache, abdominal pain, fever, and skin rash. Conclusion: Treatment with IVIG in SSc patients could be helpful and safe in patients with cutaneous, muscular, or digestive manifestations. [ABSTRACT FROM AUTHOR]

ABSTRACT Although lenalidomide is an essential treatment for multiple myeloma (MM), skin rashes are a common adverse event. This retrospective study aimed to examine the association between skin rash development during lenalidomide treatment and the prognosis of relapsed/refractory MM. All patients who received lenalidomide at 10 hospitals between July 2009 and December 2015 were included in the study. The relationship of skin rash development with disease progression and survival was evaluated. Multivariate analysis was performed to identify factors affecting disease progression or survival, including skin rash. Of the 245 patients analyzed, 70 developed skin rashes. The median progression‐free survival (PFS) of patients with skin rashes was 22.4 months, whereas the median PFS for patients who did not develop skin rashes was 10.5 months (p = 0.003). The median overall survival for patients with and without skin rash was 42.6 and 24.6 months, respectively (p = 0.013). Multivariate regression analysis showed that skin rash was an independent prognostic factor for PFS (p = 0.009). In this study, patients with skin rashes during lenalidomide treatment had significantly better PFS than those without such symptoms, indicating that lenalidomide‐associated skin rashes may be a predictor of clinical outcomes in patients with MM. [ABSTRACT FROM AUTHOR]

Background Obesity is on the rise globally in adults and children, including in tropical areas where diseases such as dengue have a substantial burden, particularly in children. Obesity impacts risk of severe dengue disease; however, the impact on dengue virus (DENV) infection and dengue cases remains an open question. Methods We used 9 years of data from 5940 children in the Pediatric Dengue Cohort Study in Nicaragua to determine whether pediatric obesity is associated with increased susceptibility to DENV infection and symptomatic presentation. Analysis was performed using generalized estimating equations adjusted for age, sex, and preinfection DENV antibody titers. Results From 2011 to 2019, children contributed 26 273 person-years of observation, and we observed an increase in prevalence of overweight (from 12% to 17%) and obesity (from 7% to 13%). There were 1682 DENV infections and 476 dengue cases in the study population. Compared with participants with normal weight, participants with obesity had higher odds of DENV infection (adjusted odds ratio [aOR], 1.21; 95% confidence interval [CI]: 1.03–1.42) and higher odds of dengue in DENV-infected individuals (aOR, 1.59; 95% CI: 1.15–2.19). Children with obesity infected with DENV showed increased odds of presenting fever (aOR, 1.46; 95% CI: 1.05–2.02), headache (aOR, 1.51; 95% CI: 1.07–2.14), and rash (aOR, 2.26; 95% CI: 1.49–3.44) when compared with children with normal weight. Conclusions Our results indicate that obesity is associated with increased susceptibility to DENV infection and dengue cases in children, independent of age, sex, and preinfection DENV antibody titers. [ABSTRACT FROM AUTHOR]

Objective: Late-onset systemic lupus erythematosus (LoSLE) is known to possess characteristics different from those of early-onset SLE (EoSLE), thereby making their diagnosis difficult. This study aimed to assess the characteristic features of LoSLE in Japan, a model country with a super-aged society. Methods: Data were obtained from the Lupus Registry of Nationwide Institutions, which includes a multicenter cohort of patients with SLE in Japan who satisfied the 1997 American College of Rheumatology revised classification criteria for SLE. Data were compared between patients with LoSLE (≥50 years old at onset) and EoSLE (<50 years old at onset). To identify factors associated with LoSLE, binary logistic regression was used for the multivariate analysis, and missing values were complemented by multiple imputations. We also conducted a sub-analysis for patients diagnosed within 5 years of onset. Results: Out of 929 enrolled patients, 34 were excluded owing to a lack of data regarding onset age. Among the 895 remaining patients, 100 had LoSLE, whereas 795 had EoSLE. The male-to-female ratio was significantly higher in the LoSLE group than in the EoSLE group (0.32 vs 0.11, p < 0.001). With respect to SLEDAI components at onset, patients with LoSLE exhibited a higher frequency of myositis (11.9% vs 3.75%, p = 0.031), lower frequency of skin rash (33.3% vs 67.7%, p < 0.001), and lower frequency of alopecia (7.32% vs 24.7%, p = 0.012). No significant differences in overall disease activity at onset were observed between the two groups. Regarding medical history, immunosuppressants were more commonly used in EoSLE. A multivariate analysis revealed that a higher male proportion and a lower proportion of new rash at onset were independent characteristic features of LoSLE. We also identified late onset as an independent risk factor for a high SDI score at enrollment and replicated the result in a sub-analysis for the population with a shorter time since onset. Conclusions: We clarified that LoSLE was characterized by a higher male proportion, a lower frequency of skin rash and a tendency to organ damage. Now that the world is faced with aging, our results may be helpful at diagnosis of LoSLE. [ABSTRACT FROM AUTHOR]

PurposeMaterials and methodsResultsConclusionsTo estimate the number of ocular syphilis (OS) cases diagnosed in a tertiary care centre in Portugal, correlate with increasing syphilis diagnoses and characterize the OS population.Retrospective, observational, single-center study that included patients diagnosed with OS between 2015 and 2023 at the local health unit of Coimbra. Demographic data were collected, and a complete ophthalmological examination was performed with multimodal imaging acquisition. Data on syphilis reports from the National System of Epidemiologic Surveillance were correlated with OS data.Fifty-four patients with OS were observed; mean age was 54.17 ± 14.46 years, 38 (70.37%) were male and 18 (47.37%) men who have sex with men. The proportion of OS in syphilis patients per year ranged from 0% to 10.34%. One-quarter were co-infected with HIV. Forty-nine patients (91.84%) complained of decreased visual acuity on presentation. Twenty-two (40.74%) had systemic findings (mostly skin rash). Two-thirds had bilateral disease and half presented with anterior segment involvement. Eighty-five percent had posterior segment involvement, mostly placoid chorioretinitis. Forty-one percent had optic nerve involvement. All patients were admitted and underwent 2-week treatment with intravenous penicillin. Visual acuity improved from logMAR 0.70 to logMAR 0.26 (p < 0.001).Ocular syphilis is a heterogeneous disease with a wide range of presentations. The incidence is on the rise and therefore OS must be considered in every patient with anterior and posterior uveitis, panuveitis and optic neuritis, with or without systemic manifestations. [ABSTRACT FROM AUTHOR]

Background: Effective management of adverse events is required to maintain sufficient imatinib dosing when treating patients with gastrointestinal stromal tumors (GISTs). Skin rash is a common adverse event of imatinib, which can be effectively controlled by systemic steroid treatment without imatinib dose modification or interruption. However, the impact of the use of systemic steroids on the efficacy of imatinib treatment remains unclear. Methods: Between October 2014 and February 2022, 277 consecutive patients from a prospective registry of GIST patients were included as the study population. Patients who started systemic steroids due to grade ≥ 3 skin rash or grade 2 skin rash with grade 2 pruritis were classified as the steroid group, whereas patients who did not develop a skin rash or those who did not require steroids for a mild skin rash were classified as the control group. Efficacy outcomes were compared between the two groups. Results: Among the 277 patients, 30 (10.8%) were treated with systemic steroids for skin rash. There was no significant difference in progression free survival (PFS) or overall survival (OS) between the steroid and control groups (3-year PFS, 67.7% vs. 65.1%, p = 0.53; 3-year OS, 91% vs. 89.9%, p = 0.67, respectively). The use of systemic steroids was not an independent factor associated with PFS (hazard ratio 0.73, 95% confidence interval 0.36–1.49, p = 0.39) and OS (hazard ratio 0.37, 95% confidence interval 0.12–1.18, p = 0.09). In the steroid group, patients who successfully maintained the imatinib dosage showed a trend toward more favorable survival outcomes than those who did not (3-year PFS, 73.3% vs. 44.4%, p = 0.34; 3-year OS, 95.8% vs. 75.0%, p = 0.15, respectively). Conclusions: The use of systemic steroids for the control of imatinib induced severe skin rash did not adversely affect the efficacy outcomes of imatinib in patients with advanced GIST. [ABSTRACT FROM AUTHOR]

Background: Chagas disease (ChD) is endemic in many parts of the world and can be transmitted through organ transplantation or reactivated by immunosuppression. Organs from infected donors are occasionally used for transplantation, and the best way of managing the recipients remains a subject of debate. Methods: We present a single‐center cohort study describing a 10‐year experience of kidney transplantation in patients at risk of donor‐derived ChD and or reactivation. Patients received prophylactic treatment with Benznidazole and were monitored for transmission or reactivation. Monitoring included assessing direct parasitemia, serology, and polymerase chain reaction (PCR). Results: Fifty‐seven kidney transplant recipients (KTRs) were enrolled in the study. Forty‐four patients (77.2%) were at risk of primary ChD infection, nine patients (15.8%) were at risk of disease reactivation, and four patients (7.0%) were at risk of both. All patients received Benznidazole prophylaxis, starting on the first day after transplantation. Parasitemia was assessed in 51 patients (89.5%), serology also in 51 patients (89.5%), and PCR in 40 patients (70.2%). None of the patients exhibited clinically or laboratory‐detectable signs of disease. A single patient experienced a significant side effect, a cutaneous rash with intense pruritus. At 1‐year post‐transplantation, the patient and graft survival rates were 96.5% and 93%, respectively. Conclusion: In this study, no donor‐derived or reactivation of Trypanosoma cruzi infection occurred in KTRs receiving Benznidazole prophylaxis. [ABSTRACT FROM AUTHOR]

The article focuses on a 15-year-old male with fever, rash, and abnormal laboratory findings, ultimately diagnosed with a viral infection. Topics include his medication history with minocycline, the broad differential diagnosis including conditions like toxic shock syndrome and Stevens-Johnson syndrome, and the positive respiratory viral panel for human metapneumovirus.

The article focuses on a 10-year-old female with a month-long history of intermittent fever, fatigue, joint pain, and rash, whose condition worsened despite outpatient evaluation, prompting a return to the pediatric emergency department. Topics include the patient's medical history including epilepsy and recent COVID-19 vaccination, laboratory findings such as anemia and a positive respiratory pathogen panel for Coronavirus OC43.

The article focuses on a 5-month-old infant with a rapidly expanding rash and fever, initially misdiagnosed as cellulitis, but later identified as a potential spider bite. Topics include her clinical presentation and progression of symptoms, the initial treatment with antibiotics, and the findings of the blackish skin lesion with expanding erythema and subsequent evaluation in the emergency department.

BACKGROUND: Amivantamab is an epidermal growth factor receptor (EGFR) and MET bispecific antibody approved for certain patients with advanced non-small cell lung cancer with EGFR variant. Cutaneous toxicities are known on-target effects of EGFR inhibition. OBJECTIVES: This article describes the occurrence and management of cutaneous toxicities in patients whose disease progressed on platinum chemotherapy treated with amivantamab. METHODS: Post hoc analysis evaluated incidence, severity, and time to first onset of rash and paronychia. Five nurses and advanced practice providers were interviewed. FINDINGS: Of 380 patients, 296 (78%) experienced treatment-related rash and/or paronychia. Paronychia (43%), rash (36%), and dermatitis acneiform (35%) were most frequent, with scalp rash reported by 17%. Treatment modifications because of rash and paronychia were infrequent. Nurses and advanced practice providers collaborate with physicians to manage cutaneous toxicities by administering comedications, modifying amivantamab dose, and educating patients. [ABSTRACT FROM AUTHOR]

The authors present a case of meningoencephalitis caused by Varicella zoster virus (VZV) infection, which initially manifested as orbital myositis followed by rapid progression to orbital apex syndrome, meningoencephalitis and death. There was no development of a cutaneous rash. An orbital biopsy demonstrated VZV infection, which was confirmed on a lumbar puncture. In this case, VZV meningoencephalitis was not responsive to steroid or antiviral therapy. This case highlights an atypical presentation of VZV with orbital myositis preceding intracranial involvement. [ABSTRACT FROM AUTHOR]

Background: Levamisole is less expensive and has a better toxicity profile compared to other steroid sparing agents used in nephrotic syndrome. It has a plasma half-life of 2.0 to 5.6 hours, but is conventionally administered on alternate days. We aimed to assess whether daily levamisole is safe and more effective than standard alternate-day therapy in maintaining remission in children with frequently relapsing or steroid-dependent nephrotic syndrome (FR/SDNS). Methods: An open-label randomized controlled trial was conducted in children with FR/SDNS. Group A received daily while Group B received alternate-day levamisole (2–3 mg/kg/dose) for 12 months. Prednisolone was tapered off by 3 months. Patients were monitored for relapses, further steroid requirement, and adverse effects. Results: A total of 190 children with FR/SDNS (94 in Group A and 96 in Group B) were analyzed. Sustained remission for 12 months was observed in 36% of Group A and 27% of Group B patients (p = 0.18). Numbers completing 12 months in the study were 67% in Group A and 56% in Group B (p = 0.13). Time to first relapse, persistent FR/SDNS, and withdrawal due to poor compliance were statistically similar in both groups, while relapse rate and cumulative steroid dosage were significantly lower in Group A compared to Group B (p = 0.03 and p = 0.02, respectively). The incidence of adverse effects was comparable in both groups, with reversible leucopenia and hepatic transaminitis being the commonest. Conclusions: Daily levamisole therapy was not superior to alternate-day therapy in maintaining sustained remission over 12 months. Nevertheless, relapse rate and cumulative steroid dosage were significantly lower without increased adverse effects. A higher resolution version of the Graphical abstract is available as Supplementary information [ABSTRACT FROM AUTHOR]

Morning Report is a time-honored tradition where physicians-in-training present cases to their colleagues and clinical experts to collaboratively examine an interesting patient presentation. The Morning Report section seeks to carry on this tradition by presenting a patient's chief concern and story, inviting the reader to develop a differential diagnosis and discover the diagnosis alongside the authors of the case. This report examines the story of a 59-year-old man who initially had a sore throat, a truncal rash, and fever. Two weeks later, arthralgias and abrupt bilateral hearing loss developed. Using questions, physical examination, and testing, an illness script for the presentation emerges. As the clinical course progresses, the differential is refined until a diagnosis is made. [ABSTRACT FROM AUTHOR]

In Ghana, widespread backyard farming on arable land with refuse waste is driven by the enriched physicochemical properties of dumpsite soils, supporting crop growth. However, the enhanced levels of heavy metals have raised concerns among policymakers focusing on the environmental challenges posed by dumpsites soil. The purpose of the study was to assess the awareness of dumpsites farmers on soil physicochemical properties and dangers posed by accumulated toxic heavy metals. A total of 100 vegetable farmers were interviewed in three communities to gauge their awareness of dumpsite soil properties and potential contamination. A semi-structured questionnaire was purposively used to solicit for information from farmers. Chi-square (χ2) test of homogeneity was used to ascertain if there was any uniformity among respondents in the different communities studied. The findings showed that the level of education of the famers had no relationship (p = 0.21) with farmers' educational level. Dumpsites farmers' knowledge on soil physicochemical properties had a significant (p = 0.02) relationship with farmers' awareness of toxic elements in dumpsites. Farmers' soil physicochemical knowledge further had a significant (p = 0.03) relationship with their awareness that, plants on dumpsites absorb toxic elements. Furthermore, ailments commonly associated with heavy metals as reported by farmers included skin rashes, cough, diarrhoea, and cholera. It can be concluded that although dumpsite farmers exhibit some awareness about the physicochemical properties of their soil and the risks associated with toxic heavy metals, there is still room for improvement. Despite education levels not significantly impacting awareness, targeted intervention programs are necessary to enhance understanding and address this pressing issue effectively. We recommend an implementation of a tailored educational programs for dumpsite farmers to enhance understanding of soil properties and heavy metal risks. There should be improved access to soil testing and interpretation to enable informed decision-making among farmers. Foster collaborations among stakeholders for sustainable waste management, soil remediation, and health monitoring to mitigate heavy metal contamination are also recommended. [ABSTRACT FROM AUTHOR]

Background: Clinical amyopathic dermatomyositis is characterized by cutaneous symptoms but lacks muscle symptoms. Anti-melanoma differentiation-associated gene 5 antibodies are frequently found in Japanese patients with clinical amyopathic dermatomyositis. Patients with rapidly progressive interstitial lung disease with positive anti-melanoma differentiation-associated gene 5 antibodies have poor prognoses, and majority of them are treated with combination immunosuppressive therapy; however, the best treatment is yet to be determined. Case presentation: A 52-year-old Asian male patient presented with a chief complaint of dyspnea on exertion. He had a typical skin rash and rapidly progressive interstitial pneumonia. Additionally, anti-melanoma differentiation-associated gene 5 antibodies were detected; therefore, he was diagnosed with dermatomyositis-associated interstitial pneumonia. Respiratory failure worsened despite administering steroid pulse therapy, tacrolimus, and cyclophosphamide. Consequently, plasma exchange was performed on day 13 of admission. After a slight improvement, the patient's respiratory failure worsened. Thus, cyclophosphamide was replaced by tofacitinib on day 28. Although respiratory failure improved and the progression of interstitial pneumonia seemed under control, βD-glucan level increased and Aspergillus antigen was detected on day 49. Micafungin and voriconazole were administered, but the patient succumbed to worsening respiratory failure on day 61. The pathological autopsy revealed multiple nodular lesions with cavity formation in both lungs and the presence of Aspergillus with severe neutrophilic infiltration and necrosis, which supported the diagnosis of invasive pulmonary aspergillosis. Conclusion: The patient with anti-melanoma differentiation-associated gene 5 antibody-related rapidly progressive interstitial lung disease, whose disease was difficult to control after the administration of triple immunosuppressive therapy (steroids, tacrolimus, and cyclophosphamide), showed good response with tofacitinib. Unfortunately, the patient died of invasive pulmonary aspergillosis owing to severe immunosuppression; thus, the signs of complications should be promptly detected. [ABSTRACT FROM AUTHOR]

Background: Multisystem Inflammatory Syndrome in Children (MIS-C) is a rare complication, which develops within 3–6 weeks after SARS-CoV2 infection. The coronavirus disease 2019 (COVID-19) vaccine was firstly introduced in adults and adolescents and later in patients aged 5–11 years old. Although a reduced incidence of MIS-C and with less severe symptoms has been reported in vaccinated adolescents, there is little knowledge in children younger than 12 years of age. In addition, it is not understood whether MIS-C in vaccinated patients can be triggered by Covid19 vaccination or be secondary to a recent asymptomatic Sars-Cov2 infection. Case presentation: We describe the case of a Caucasian 6-year-old girl, one month after double COVID-19 vaccination, who presented fever, acute abdominal pain, rash, pharyngotonsillitis, cheilitis, cervical lymphadenopathy without a prior detected Sars-Cov2 infection. She also had lymphopenia, increase in inflammatory markers, cardiac and pulmonary involvement. Therefore, we dosed both anti Sars-Cov2 Spike and Nucleocapsid antibodies, which were positive and allowed us to confirm the diagnosis of MIS-C. We promptly administered intravenous immunoglobulins and methylprednisone, resulting in the initial regression of fever. During the hospitalization, the child also developed pancreatitis and severe neurological involvement, including irritability, drowsiness, distal tremor, dyskinesia and buccal asymmetry with complete resolution after 2 months. After 3 months from the onset of the symptoms, she reported a transient loss of hair compatible with telogen effluvium. After 12 months of follow-up, she did not show any symptomatic sequelae. Conclusions: This case raises the question of whether COVID-19 vaccination may be involved in the pathogenesis of MIS-C in children between the ages of 5 and 11 years old. [ABSTRACT FROM AUTHOR]

Objectives: Yao syndrome (YAOS, OMIM# 617321) is a kind of systemic autoinflammatory diseases (SAIDs) linked to the nucleotide-binding oligomerization domain containing 2 (NOD2). Clinical reports of YAOS in China are sparse. Herein, we reported the largest YAOS cohort of Chinese patients to expand the understanding of its phenotype, genotype, and therapeutic responses. Methods: This study enrolled 15 adult patients diagnosed with YAOS at Peking Union Medical College Hospital from April 2015 to May 2024. Whole-exome sequencing was performed on all patients. Clinical data, genetic variations, and treatment responses were documented and compared with a Caucasian cohort. Results: The mean age of disease onset was 35 ± 17 years old. The most common clinical manifestations included recurrent high-grade fever (100%), gastrointestinal symptoms (73.3%), arthralgia/arthritis, fatigue, myalgia, and lower extremity swelling (46.7%). All patients exhibited elevated acute-phase reactants during episodes. 12 heterozygous NOD2 variants were identified, with Q902K in 4 patients, R471C in 3, and variants c.-14C>T, A110T, S127L, R311W, A432V, Y514H, R541P, A661P, K818Q, A886V each found in individual patients. 90% of the patients responded well to glucocorticoids, and 55.6% to sulfasalazine. 66.7% of patients who received TNF inhibitors achieved complete resolution of symptoms. Additionally, one patient each responded favorably to canakinumab and tocilizumab. Compared to the Caucasian cohort, our cohort exhibited a more balanced gender ratio and a higher proportion of recurrent fever, proteinuria/hematuria as well as more frequent leukocytosis, elevated acute phase reactants, and anemia. Lower proportions of arthralgia/arthritis, skin rashes, headaches, and sicca-like symptoms were noted in our cohort. Moreover, a higher proportion of patients in our cohort showed a good response to TNF inhibitors. Conclusion: Chinese patients with YAOS had more pronounced inflammatory manifestations compared to the Caucasian cohort. Variants c.-14C>T, A110T, S127L, A661P, K818Q, A886V, R471C, and A432V were identified as novel NOD2 variants in YAOS. TNF, IL-6, and IL-1 inhibitors are the promising treatment options. These findings expand the clinical spectrum, genetic profile, and treatment efficacy of YAOS, underscoring the need for heightened awareness of this disease in diverse populations. [ABSTRACT FROM AUTHOR]

Background: Mpox, is a zoonosis that is known to be endemic in several Central and West African countries. Recently, in 2022, it has emerged in Europe and United States, what raised the alarm to be declared in late June 2024 as a public health event of international concern. This study aimed to give insight about the recent spread of mpox in Sudan, and documents the epidemiologic situation. Methods: Through a cross-sectional design, Sudan mpox data was extracted from the disease surveillance line-list at the national level at Sudan Federal Ministry of Health. the data was customized and then analyzed using Epi Info7 software. Analysis was done using frequencies and percentages and the results presented in tables and figures. Permission and ethical approval were obtained from the Health Emergency and Epidemic Control Directorate at the Federal Ministry of Health. Results: The outbreak of mpox was confirmed after testing of initial specimens outside Sudan with positivity rate of 72%. Later the cases continued to be reported based on the clinical diagnosis and standard case definition. Out of 375 reported cases, 54.4% were males, while 45.6% were females. The age of cases ranged from one month to 78 years with majority (41.1%) of the cases were children under 5 years of age. Regarding the reported symptoms, all cases had the characteristic skin rash and 74.1% of them had fever. Other symptoms included, headache (31.5%), sore throat (30.9%) and lymphadenopathy (26.1%). For occupation, 35.7% were preschool and 10.4% were school children, 9% of cases were prisoners. Around 22 (5.8%) reported contact history with a confirmed case, while (5.6%) of the cases were imported cases. Cases were reported from 17 states with 42 affected localities (districts) with an overall attack rate of 2.36/ 100,000. The highest number of cases was reported from Gadaref (45.3%), West Darfur (25.9%), Khartoum (13.3%) and north Darfur (3.5%). In Gadaref, 146 (85.8%) of the cases were from a refugees' camp. Started in epi week 19, the outbreak peaked in week 38 and last in week 42. Conclusion: Mpox was confirmed in the new Sudan for the first time with cases reported in most of states. Although importation of the virus is hypothesized, internal hidden circulation is possible and more in-depth investigation is highly recommended. The higher rate of infection among preschool, school children and refugees, highlights the need to strengthen the prevention and control measures in schools and camps. More focus on the data completeness is required for better understanding of the disease and can be ensured by the surveillance directorate through training of staff and updating of reporting forms. Strengthening the lab capacity inside the country is a necessity to ensure testing of all the clinically diagnosed cases. [ABSTRACT FROM AUTHOR]

Background: Nivolumab, an immune checkpoint inhibitor used to treat several malignancies, is associated with immune-related adverse events (IrAEs). Original dosing for melanoma was 3 mg/kg (maximum 240 mg) every 2 weeks (Q2W). Based on simulation studies depicting similar efficacy and toxicity to original dosing, extended interval dosing of 6 mg/kg (maximum 480 mg) every 4 weeks (Q4W) was introduced. Objective: This study will compare safety between Q2W and Q4W dosing at BC Cancer in melanoma patients. Methods: Retrospective chart review for reported incidence, onset, and severity of IrAEs in melanoma patients treated with nivolumab Q2W and Q4W dosing was completed. Fisher's test was conducted for first incidence IrAEs using Microsoft Excel. Results: Seventy-one patients were identified (Q2W n = 35, Q4W n = 36). Baseline characteristics were similar in both groups. No statistically significant difference was found in incidence of IrAEs between Q2W and Q4W dosing (Q2W 40% vs Q4W 50%, p = 0.477). Rash was most common (Q2W 79% vs Q4W 50%) followed by hypothyroidism (Q2W 33% vs Q4W 20%). Median onset of IrAEs seemed later with Q4W dosing (Q2W cycle 1 vs Q4W cycle 4). Regardless of dosing, most IrAEs were grade 1–2 in severity (Q2W 100% vs Q4W 89%). Conclusion: Q4W dosing is associated with comparable incidence and potentially later onset of IrAEs compared to Q2W dosing. Most IrAEs in both dosing groups were similar and mild. Therefore, Q4W dosing offers a safe alternative to Q2W dosing while providing benefits including decreased workload for staff, decreased clinic visits, and viral exposure by patients. [ABSTRACT FROM AUTHOR]

Background: Inborn errors of immunity (IEI), formerly referred to as primary immunodeficiencies, manifest with a wide range of symptoms such as increased susceptibility to infections, immune dysregulation, and autoinflammation. Although most cases manifest in childhood, onset during the neonatal period is rare but potentially critical. Summary: In this review, we discuss the diverse clinical presentations of IEI and the specific challenges they pose to neonatologists. Rather than detailing every molecular defect, we focus on common clinical scenarios in neonates and young infants, providing practical diagnostic strategies to ensure timely and effective therapeutic interventions. Key Messages: Clinical presentations of IEI in neonates may include delayed separation of the umbilical cord, skin rashes such as eczema and erythroderma, and recurrent episodes of inflammation. We also highlight immunological emergencies that require urgent medical attention, such as hyperinflammatory activity mimicking acute neonatal liver failure, sometimes seen in hemophagocytic lymphohistiocytosis. We also discuss appropriate medical action in the case of a positive newborn screening for severe T-cell defects. Early medical intervention in such circumstances may significantly improve outcomes. [ABSTRACT FROM AUTHOR]

The present case series examined five instances of psoriasiform drug eruption diagnosed between 2014 and 2022 at the study site and 23 cases of drug eruption manifesting psoriasiform lesions which had been reported between 1986 and 2022. The causative drug, distribution of the skin eruptions, clinical latency to eruption, treatment course, and histopathological findings were investigated. The most common causative agents were calcium channel blockers (CCB) (64.5%). Of the 28 cases of psoriasiform drug eruption for which details of the eruption sites were reported, 46.4% occurred on the face, which was slightly higher than the usual distribution of psoriasis. CCB were responsible for 80.0% of the cases of facial skin rash. The mean time from the administration of the suspected drug to eruption onset was 25.0 months (range: 0.5–120 months; median: 13.0 months). In all the cases, the skin rash improved after the causative drug was discontinued. CCB were the most common causative agent, and the eruptions more commonly occurred on the face than in normal psoriasis, suggesting that it is especially important to confirm whether there is a history of CCB administration in psoriasis patients with extensive, facial skin eruptions. [ABSTRACT FROM AUTHOR]

Measles is a highly contagious but vaccine-preventable airborne-transmitted viral infection of which there has been a recent resurgence of cases worldwide over the past year, including in countries such as the UK, which had previously successfully achieved endemic measles elimination through vaccination programmes. Measles is typically a self-limiting illness, but can rarely cause severe, life-threatening disease, particularly when complicated by respiratory or neurological involvement. These severe complications are not typically seen in the absence of immunosuppression. We describe a rare case of severe measles with pneumonitis in an immunocompetent adult necessitating admission to an intensive care unit (ICU). [ABSTRACT FROM AUTHOR]

Objective: To determine the prevalence and factors associated with self-medication among students with medical and non-medical backgrounds in Peshawar, Khyber Pakhtunkhwa. Study Design: Cross-sectional study. Setting: Medical and Non-medical Universities of Peshawar. Period: May to October 2022. Methods: For this comparative analysis, sample size was calculated based on the formula of Cochran, taking a 95% confidence level with 5% precision. The sampling was conducted using a non-probability convenience approach. Data was collected using a structured questionnaire and analyzed with the help of SPSS v.25. Results: A study sample of 427 participants with a 95% response yielded 212 medical students and 215 non-medical students. The prevalence of self-medication among medical and non-medical students was 35.36% and 39.35%, respectively. The occurrence of self-medication was reported as 7% daily, 11% weekly, 11% fortnightly, and 71% monthly. Among the reasons for self-medication, headache was the most common (49.65%), followed by cough/sore throat/cold (31.85%), fever (26.46%), generalized body aches (17.56%), heartburn/indigestion (14.75%), nausea/vomiting (9.36%), constipation (4.91%), anxiety/depression (4.68%), and insomnia (3.51%), while 8.89% constitutes other factors like allergies, skin rashes, and weight loss. The main reason for self-medication was the mild nature of the illness, which accounted for 31.85% of cases. Conclusion: The study indicated a considerable number of student population involved in self-medication practices. The prevalence of this behavior was slightly more common among non-medical students than medical students. Various factors contributed to self-medication ranging from physical symptoms to mental health issues. [ABSTRACT FROM AUTHOR]

Among the occupational skin diseases, bird mite dermatitis (gamasoidosis) is an infrequent and under-reported condition. A report is presented of a group of workers employed in a habitat restoration centre who developed episodes of pruritus and skin rashes associated with bird mites. The mite bites resolved approximately two weeks after each episode, without specific medical intervention and despite ongoing work activities. A workplace health-risk assessment and microscopic analysis of 'swabs' of the work environment aided the identification of bird mite as the causative agent. The source of the bird mites was infested red-winged starlings nesting in the roof of the workplace facility. Incidents of gamasoidosis as described are considered occupational dermatoses since they were acquired in the workplace and the diagnosis was based on clinical--parasitological analysis. When diagnosing gamasoidosis, the clinical history and physical examination provide useful clues, but the clinical features of skin lesions of arthropod or other insect bites are very similar. Microscopic detection of the mite is confirmatory. [ABSTRACT FROM AUTHOR]

Introduction: Incorporation of AKT inhibitors into adjuvant therapy for advanced or metastatic breast cancer has improved clinical outcomes. However, the safety of AKT inhibitors should be better evaluated, given the possibility of prolonging survival and impacting patient quality of life. Our aim was to assess how the addition of AKT inhibitors to adjuvant therapy affects treatment-related adverse events. Methods: We evaluated binary outcomes with risk ratios (RRs), with 95% confidence intervals (CIs). We used DerSimonian and Laird random-effect models for all endpoints. Heterogeneity was assessed using I2 statistics. R, version 4.2.3, was used for statistical analyses. Results: A total of seven RCTs comprising 1619 patients with BC. The adverse effects that show significance statistical favoring the occurrence of adverse effects in AKT inhibitor were diarrhea (RR 3.05; 95% CI 2.48–3.75; p < 0.00001; I2 = 49%), hyperglycemia (RR 3.4; 95% CI 1.69–6.83; p = 0.00058; I2 = 75%), nausea (RR 1.69; 95% CI 1.34–2.13; p = 0.000008; I2 = 42%), rash (RR 2.79; 95% CI 1.49–5.23; p = 0.0013; I2 = 82%), stomatitis (RR 2.24; 95% CI 1.69–2.97; p < 0.00001; I2 = 16%) and vomiting (RR 2.99; 95% CI 1.85–4.86; p = 0.00009; I2 = 42%). There was no significant difference between the groups for alopecia (p = 0.80), fatigue (p = 0.087), and neuropathy (p = 0.363380). Conclusion: The addition of AKT inhibitors to adjuvant therapy was associated with an increase in treatment-related adverse events. These results provide safety information for further clinical trials evaluating AKT inhibitor therapy for patients with metastatic BC. Clinicians should closely monitor patients for treatment-related adverse events to avoid discontinuation of therapy and morbidity caused by these early-stage therapies. [ABSTRACT FROM AUTHOR]

Introduction The purpose of this study is to examine the relationship between subjects' oral health characteristics and the incidence of a dental emergency over 1 year. Materials and Methods The Corporate Dental System (CDS) generated a limited data set for service members assigned for dental care at Fort Liberty, North Carolina. Data extracted included demographics and oral health characteristics (dental readiness classification, caries risk, periodontal screening and recording values (PSR), and tobacco use). Subjects were grouped based on the presence of a dental emergency visit code in the past year. Results A total of 20,210 individuals were included in the sample; 8.5% of individuals sought emergency dental care during the period. Kruskal-Wallis tests found significant differences (P  < 0.05) in dental emergencies between age groups, sex, rank, dental readiness classification, and caries risk. Younger age, female soldiers, and low caries risk had the most significant increase in dental emergencies. There was no significant difference in dental emergencies between PSR or tobacco usage groups. Conclusion This study supports a relationship between demographics, oral health characteristics, and dental emergencies. Age may be related to service members entering the military with untreated or managed dental needs, notably around the age of third molar eruption. Sex differences may be related to willingness or preference to utilize non-emergency dental care visits. The inverse relationship between caries risk and dental emergencies could be due to identification and management of high caries risk patients. This project highlights the need for further study and increasingly discrete measurement of oral health care characteristics and coding for etiologies of dental emergencies. [ABSTRACT FROM AUTHOR]

Dermatomyositis (DM) is associated with interstitial lung disease (ILD) and malignancy. However, the coexistence of ILD and malignancy (DM–ILD–malignancy) is rare, and limited information exists regarding its management. Herein, we report the case of a 70-year-old man who developed DM with rapidly progressive ILD and advanced gastric cancer and provide a literature review of managing DM–ILD–malignancy. The patient presented with typical DM skin rashes and shortness of breath, which worsened within 1 month, without muscular symptoms. Additionally, the patient tested negative for myositis-specific autoantibodies (MSAs). Computed tomography revealed ILD and advanced gastric cancer, which was confirmed on endoscopic examination to be a poorly differentiated adenocarcinoma. Although the patient's ILD progressed rapidly, surgical treatment of the cancer was prioritized. Prednisolone (PSL) 0.5 mg/kg was initiated 3 days before surgery and increased to 1 mg/kg at 7 days postoperative. Remarkable improvement in the skin rash and ILD was observed, and the PSL dose was tapered without immunosuppressants. A literature review revealed that anti-melanoma differentiation-associated gene 5 and anti-aminoacyl transfer RNA synthetase antibodies are the predominant MSAs in DM–ILD–malignancy, and the optimal treatment should be determined based on several factors, including ILD patterns, and malignancy type and stage. In particular, lung cancer may be a risk factor for the acute exacerbation of ILD, and preceding immunosuppression would be useful. Furthermore, prioritizing surgery for gastric cancer is effective because of its paraneoplastic nature. [ABSTRACT FROM AUTHOR]

Chikungunya fever, a viral illness transmitted to humans through the bites of infected mosquitoes, presents with symptoms such as high fever, severe myalgia, headache, arthralgia, rash, and vomiting. This disease predominantly manifests in Southeast Asia, Africa, and Central and South America, with a limited occurrence in Northeast Asia. To date, no such a documented case has been reported in South Korea. Herein, we present the first adolescent case of chikungunya fever in South Korea following a travel to Bali, Indonesia.

Background: In relapsed/refractory (R/R) diffuse large B‐cell lymphoma (DLBCL), a negative prognosis is frequently linked to heightened epigenetic heterogeneity. Chidamide, a selective histone deacetylase inhibitor, shows promise as a targeted therapy for R/R DLBCL by targeting abnormal epigenetic changes associated with poor prognosis. Methods: A cohort of 27 ineligible patients with R/R DLBCL participated in an open — label, single — arm study. Chidamide was administered orally at a dose of 30 mg twice weekly for one week during the induction monotherapy phase. The subsequent combination therapy phase involved oral chidamide at a dose of 20 mg twice weekly for two weeks, followed by a one‐week discontinuation period, in conjunction with intravenous R‐GDP every 21 days. Results: Among the cohort of 31 patients who underwent screening (median age: 67 years), 27 were ultimately included in the study, with 14 individuals successfully completing six cycles of C‐R‐GDP treatment. The overall best objective response rate was determined to be 79.1% (95% CI: 75.1%–83.3%), comprising a complete response rate of 45.8% (95% CI: 41.6%–49.9%) and a partial response rate of 33.3% (95% CI: 29.3%–37.4%). Within the subgroup of 14 patients who completed the full treatment regimen, the best objective response rate reached 100%, with 71.4% achieving complete response (n = 10) and 28.6% achieving partial response (n = 4). The median follow‐up period for these patients was 17.0 months, ranging from 3.5 to 55 months. Progression‐free survival was 5.9 months and overall survival was 48.3 months. Anemia was the most common adverse event, affecting all patients. Thrombocytopenia led to treatment interruption or dose reduction in 13 patients. Other common adverse events included hypocalcemia, hyponatremia, and hypokalemia. Three patients experienced grade 3 pneumonitis and one had grade 3 skin rash. Conclusions: Chidamide combined with R‐GDP is a safe and effective treatment option for patients with R/R DLBCL who are not eligible for autologous stem cell transplantation. [ABSTRACT FROM AUTHOR]

Background: Non-metastatic castration-resistant prostate cancer (nmCRPC) is an asymptomatic condition with the potential to progress to metastasis. Novel hormonal agents (NHAs) are currently considered the gold standard treatment for nmCRPC, offering significant survival benefits. However, further evidence is needed to determine whether there are differences in the performance of these drugs among Asian populations. Methods: This retrospective analysis of nmCRPC patients aims to compare the efficacy and safety of three NHAs–apalutamide, darolutamide, and enzalutamide. Data were collected from two prominent prostate care centers in Taichung, Taiwan. Patient characteristics, treatment details, PSA responses, and adverse events were analyzed. Statistical comparisons were performed, and the study received Institutional Review Board approval. Results: Total of 64 patients were recruited in this study, including 29 darolutamide, 26 apalutamide, and 9 enzalutamide patients. Baseline characteristics varied between the three patient groups, but the treatment response still revealed similar results. The apalutamide group experienced more adverse events, notably skin rash. Discontinuation rates due to adverse events differed among the groups, and patients receiving darolutamide were less likely to discontinue treatment. Conclusion: This real-world study provides insights into NHA utilization in nmCRPC within the Taiwanese population. Adverse event profiles varied, emphasizing the need for individualized treatment decisions. The study underscores the importance of regional considerations and contributes valuable data for optimizing treatment outcomes in nmCRPC. [ABSTRACT FROM AUTHOR]

aiman,1 Khalid A Kheirallah,2 Ahmad Alrawashdeh,3 Tareq Saleh,4 Maha Obeidat,5 Yareen J Alawneh,2 Ziydoun Abu Sanad,5 Wajdi Amayreh,1 Rama J Alawneh21Department of Pediatrics, Faculty of Medicine, Yarmouk University, Irbid, Jordan; 2Department of Public Health and Family Medicine, Faculty of Medicine, Jordan University of Science and Technology, Irbid, Jordan; 3Department of Allied Medical Sciences, Faculty of Applied Medical Sciences, Jordan University of Science and Technology, Irbid, Jordan; 4Department of Pharmacology and Public Health, Faculty of Medicine, The Hashemite University, Zarqa, Jordan; 5Department of Pediatrics, Princess Rahma Teaching Hospital, Irbid, Jordan Correspondence: Jomana W Alsulaiman, Associate Professor of Pediatrics, Department of Pediatrics, Faculty of Medicine, Yarmouk University, Irbid, 21163, Jordan, Tel + 962 07 9941 2277, Email [email protected] Background: Implementing allergy testing among children with a reported history of penicillin allergy could be challenging, particularly in developing countries with limited resources. This study screened and risk-stratified the likelihood of true penicillin allergy among children labeled with penicillin allergy in Jordan. Methods: A web-based survey, completed by parents, assessed history, type, and severity of penicillin allergic reactions, including age at diagnosis, symptoms, time to the reaction, reaction's course and resolution, and received medical evaluation/testing. Low-risk allergic symptoms were defined as vomiting, diarrhea, headache, dizziness, itching, rash, cough, or runny nose without evidence of anaphylaxis or severe cutaneous reactions. Results: A total of 530 parents of "penicillin allergy"-labeled children completed the survey. Of these, 86.4% reported allergic reactions to penicillin and 13.6% reported avoidance of penicillin due to family history. Among the former, 52.2% were male, 67.3% were three years old or younger when the reported reaction was established, and 68.3% experienced exclusively low-risk symptoms. Overall, skin rash was the most reported symptom (86.0%). High-risk symptoms were reported in 31.5% of children. About two-thirds (64.0%) of children were reported to have experienced symptoms after the first exposure to penicillin. The most common indication for antibiotic use was a throat infection (63.8%). Asthma comorbidity was significantly higher among high-risk (24.8%) compared low-risk group (11.5%). Conclusion: In Jordan, many parent-reported penicillin allergic reactions seem to be clinically insignificant and unlikely to be verifiable, which can adversely affect patients' care and antimicrobial stewardship. An appropriate clinical history/evaluation is a key step in identifying true immunoglobulin E-mediated allergic reactions and risk stratifying patients for either de-labeling those with obviously non‐immune–mediated reactions or identifying candidates for direct oral challenge test. [ABSTRACT FROM AUTHOR]

Kimura disease (KD) is a rare, chronic, inflammatory disorder of unknown etiology characterized by the development of subcutaneous lymphoid masses, usually in the head and neck region; regional lymphadenopathy; peripheral eosinophilia; and elevated levels of serum IgE. KD has been described in China and Japan as "eosinophilic hyperplastic lymphogranuloma" or "atypical granulation associated with hyperplastic abnormalities in the lymphoid tissue". The precise prevalence and incidence and the pathogenesis of KD are unknown. Trauma, infection, an IgE-mediated hypersensitivity reaction, or autoimmune processes have been postulated as possible causes. In this article, we aimed to present a patient who was investigated with elevated serum total IgE and eosinophils and diagnosed as Kimura disease in the light of the current literature. [ABSTRACT FROM AUTHOR]

Acute generalized exanthematous pustulosis (AGEP) is a sudden-onset, severe and rare adverse skin reaction characterized by nonfollicular sterile pustules tending to intertriginous localization. Lesions develop on erythematous and edematous skin. It is often triggered by drugs. This article presents a case diagnosed with AGEP due to etodolac. To the best of our knowledge, this is the second case of AGEP due to etodolac in the literature. A 47-year-old female patient presented with diffuse erythema on the extremities, a purple purpuric rash on the extensor face of both legs, and millimetric pustules on an erythematous base on the inner surface of the left arm. She stated that the reaction developed after taking 3 medications, including etodolac tablets, and gargling the throat with povidone-iodine. The patient was diagnosed with AGEP and her EuroSCAR AGEP Validation Score was calculated as 9 suggesting a definitive diagnosis of AGEP. All of the suspected drugs were discontinued. Methylprednisolone 16 mg/day, a local corticosteroid, and an oral antihistamine were started. Her symptoms resolved and laboratory abnormalities returned to normal within 2 weeks. Patch tests were performed 3 months after the reaction with the suspected drugs. The patch testing showed that only 10% etodolac at 48-, 72- and 96-hour readings were positive (++). The patient was diagnosed with AGEP due to etodolac. AGEP is often secondary reaction to drugs. The most frequent causative drugs are diltiazem, aminopenicillins, pristinamycin, terbinafine, sulphonamides, quinolones, and hydroxychloroquine. AGEP secondary to NSAIDs is very rare. Only one previous case of AGEP due to etodolac was reported in 2011. [ABSTRACT FROM AUTHOR]

Background: Levamisole is a commonly used steroid-sparing agent (SSA), but the reported incidence of antineutrophil cytoplasmic antibody (ANCA) positivity has been concerning. Methods: Observational cross-sectional study wherein children aged 2 to 18 years with frequently relapsing/steroid dependent nephrotic syndrome (FRNS/SDNS) on levamisole for ≥ 12 months were tested for ANCA. Results: A total of 210 children (33% female), median age of 7.3 (IQR: 5.6–9.6) years, and a median duration of levamisole exposure of 21 (IQR: 15–30) months were tested. ANCA was positive in 18% (n = 37): 89% (n = 33) perinuclear ANCA (pANCA), 3% (n = 1) cytoplasmic ANCA (cANCA), and 8% (n = 3) both. Of ANCA-positive children, none had reduced eGFR or abnormal urinalysis. The majority of these children were asymptomatic (81%, n = 30). Rash was more common among ANCA-positive children [6/37 (16%) vs. 3/173 (2%), p = 0.0001]. On multivariate analysis, higher age (OR = 1.02, [95th CI: 1.01 to 1.03], p = 0.007) and longer duration of levamisole exposure (OR = 1.05, [95th CI: 1.02 to 1.08], p = 0.0007) were associated with ANCA positivity. Levamisole was stopped in ANCA-positive children with the resolution of any clinical manifestations if present. Repeat ANCA testing was performed in 54% (20/37), and all were ANCA negative by 18 months. Conclusions: Children with FRNS/SDNS on longer duration of levamisole were associated with increasing prevalence of ANCA positivity, but most of these children were clinically asymptomatic. Prospective studies are required to determine the chronology of ANCA positivity and its clinical implication. [ABSTRACT FROM AUTHOR]

Objectives To compare clinical characteristics, including the frequency of cutaneous, extramuscular manifestations and malignancy, between adults with anti-synthetase syndrome (ASyS) and DM. Methods Using data regarding adults from the MYONET registry, a cohort of DM patients with anti-Mi2/-TIF1γ/-NXP2/-SAE/-MDA5 autoantibodies, and a cohort of ASyS patients with anti-tRNA synthetase autoantibodies (anti-Jo1/-PL7/-PL12/-OJ/-EJ/-Zo/-KS) were identified. Patients with DM sine dermatitis or with discordant dual autoantibody specificities were excluded. Sub-cohorts of patients with ASyS with or without skin involvement were defined based on presence of DM-type rashes (heliotrope rash, Gottron's papules/sign, violaceous rash, shawl sign, V-sign, erythroderma, and/or periorbital rash). Results In total 1054 patients were included (DM, n  = 405; ASyS, n  = 649). In the ASyS cohort, 31% (n  = 203) had DM-type skin involvement (ASyS-DMskin). A higher frequency of extramuscular manifestations, including Mechanic's hands, Raynaud's phenomenon, arthritis, interstitial lung disease and cardiac involvement differentiated ASyS-DMskin from DM (all P  < 0.001), whereas higher frequency of any of four DM-type rashes—heliotrope rash (n  = 248, 61% vs n  = 90, 44%), violaceous rash (n  = 166, 41% vs n  = 57, 9%), V-sign (n  = 124, 31% vs n  = 28, 4%), and shawl sign (n  = 133, 33% vs n  = 18, 3%)—differentiated DM from ASyS-DMskin (all P  < 0.005). Cancer-associated myositis (CAM) was more frequent in DM (n  = 67, 17%) compared with ASyS (n  = 21, 3%) and ASyS-DMskin (n  = 7, 3%) cohorts (both P  < 0.001). Conclusion DM-type rashes are frequent in patients with ASyS; however, distinct clinical manifestations differentiate these patients from classical DM. Skin involvement in ASyS does not necessitate increased malignancy surveillance. These findings will inform future ASyS classification criteria and patient management. [ABSTRACT FROM AUTHOR]

Introduction: Holocarboxylase synthetase deficiency (HLCS deficiency, OMIM #253270) is an exceedingly rare metabolic disorder resulting in multiple carboxylase deficiencies owing to impaired biotin cycle. Clinical manifestations include severe metabolic acidosis, hyperammonemia, tachypnea, skin rash, alopecia, feeding problems, hypotonia, developmental delay, seizures, and, in severe cases, death. Methods and Results: An 8‐day‐old female neonate presented with severe lactic acidosis, necessitating sedation and mechanical ventilation. Despite receiving supportive care, no evident clinical improvement was observed, accompanied by the onset of generalized ichthyosis. Genetic analysis of actionable metabolic disorders revealed compound heterozygous variants of HLCS (NM_000411.8), specifically c.[710T>C (p.Leu237Pro)]; [1544G>A (p.Ser515Asn)], prompting the initiation of biotin mega‐dose therapy (10 mg/day). Remarkably, dramatic clinical improvement in lactic acidosis was observed the day after initiating biotin administration, leading to the discontinuation of mechanical ventilation within 6 days. The patient remained in stable condition during follow‐up, exhibiting normal growth and development along with consistently stable laboratory findings up to 18 months of age. Conclusion: Our case highlights the significance of early genetic testing in neonates with unexplained metabolic disorders to enable timely diagnosis and therapy initiation. Biotin therapy has demonstrated remarkable efficacy in improving the clinical condition of patients with HLCS deficiency, leading to favorable outcomes. [ABSTRACT FROM AUTHOR]

Hand, foot, and mouth disease (HFMD) is a common infectious disease caused by enteroviruses. Coxsackievirus A6 (CV-A6)-associated HFMD has recently emerged as a predominant disease worldwide. Here, we describe five HFMD cases caused by CV-A6 in Japan from 2019 to 2022. All clinical courses were not severe and were self-limited, and the skin exanthema with vesicles differed from that in classical HFMD. Phylogenetic analysis showed that the major epidemic strain cluster of CV-A6 was formed independently in 2011, and our latest CV-A6 strains in Japan were detected within this cluster. The five cases described in this report indicate the recent shift in the predominant and continuous disease manifestation of CV-A6-associated HFMD. [ABSTRACT FROM AUTHOR]

Background: Cutaneous manifestations of drug‐induced type IV reactions vary widely, with symmetrical drug‐related intertriginous and flexural exanthema (SDRIFE) being a less common presentation. Corticosteroids (CS), primarily known for their anti‐inflammatory effects, rarely induce hypersensitivity reactions. Objective: The aim of this case series is to report four cases of SDRIFE following systemic prednisolone therapy and to review existing CS classification proposals to better understand cross‐reactivity of CS. Patients/Methods: Patients recruited at a German dermatology centre underwent allergologic evaluation including prick and patch testing with various CS. Positive cases underwent oral challenge testing with alternative agents. The classification systems of Coopman et al. and Baeck et al. were taken into account. Discussion: Despite a paucity of literature, CS‐induced type IV reactions do occur, including SDRIFE. Classification systems based on chemical structure provide insight into cross‐reactivity patterns. Provocation tests with alternative CS highlight the complexity of managing CS hypersensitivity. Conclusion: SDRIFE may develop following systemic prednisolone therapy. Classification systems are helpful in understanding cross‐reactivity and help in the selection of alternative preparations but are not always reliable. Individualised assessment is crucial for managing CS hypersensitivity, with consideration of alternative agents and emergency use of CS when necessary. [ABSTRACT FROM AUTHOR]