Erythema Multiforme-Like Fixed Drug Eruption During Azathioprine and Hydroxychloroquine Treatment for Systemic Lupus Erythematosus Mimicking Rowell Syndrome: A Rare and Challenging Clinical Scenario.

Background. Fixed drug eruption and Rowell syndrome stand as intriguing entities with overlapping clinical and pathological features. Case Presentation. A 32-year-old female patient presented with a tender and pruritic rash on the left upper chest for 3 days. Clinical examination revealed a flaring rash on the chest, under her left eye, tongue, and lips. The patient had a significant past medical history of systemic lupus erythematous with positive (ANA, Sm, dsDNA, ribosomalP, RNP) antibodies, hypocomplementemia, inflammatory arthritis, discoid lupus, leukopenia, thrombocytopenia, and immune thrombocytopenic purpura, and avascular necrosis affecting both hips and her right knee. At the time of presentation, the patient was on azathioprine 150 mg daily and hydroxychloroquine 200 mg twice daily. Skin biopsy of the left upper chest revealed interface dermatitis with necrotic keratinocytes at the dermal-epidermal junction. Superficial and, in some areas, deep perivascular and peri adnexal lymphocytic infiltrates were observed, accompanied by eosinophils. CD123 staining highlighted 16% of the inflammatory cells. Direct Immunofluorescence for IgG, IgA, IgM, C3, and fibrinogen revealed positive linear basement membrane staining for IgG and fibrinogen, with no significant staining for the remaining immunoreactants. Considering the patient's history of medicine usage, and negative SS-A and SS-B antibody, a fixed drug eruption was favored. Discussion. This article discusses the clinical presentations, pathophysiological mechanisms, and diagnostic criteria for fixed drug eruption and Rowell syndrome. Conclusion. Awareness of the distinct clinical and histopathologic features of fixed drug eruption and Rowell syndrome, particularly when sharing cutaneous manifestations, underscores the importance of a comprehensive diagnostic approach and laboratory testing.

Socioeconomic deprivation and racialised disparities in competitive athletes with sudden cardiac arrest from the USA.

OBJECTIVE: To explore the association of socioeconomic deprivation and racialised outcomes in competitive athletes with sudden cardiac arrest (SCA) in the USA. METHODS: SCA cases from the National Center for Catastrophic Sports Injury Research (July 2014 to June 2021) were included. We matched Area Deprivation Index (ADI) scores (17 metrics to grade socioeconomic conditions) to the 9-digit zip codes for each athlete's home address. ADI is scored 1-100 with higher scores indicating greater neighbourhood socioeconomic deprivation. Analysis of variance was used to assess differences in mean ADI by racial groups. Tukey post hoc testing was used for pairwise comparisons. RESULTS: 391 cases of SCA in competitive athletes (85.4% male; 16.9% collegiate, 68% high school, 10.7% middle school, 4.3% youth) were identified via active surveillance. 79 cases were excluded due to missing data (19 race, 60 ADI). Of 312 cases with complete data, 171 (54.8%) were white, 110 (35.3%) black and 31 (9.9%) other race. The mean ADI was 40.20 (95% CI 36.64, 43.86) in white athletes, 57.88 (95% CI 52.65, 63.11) in black athletes and 40.77 (95% CI 30.69, 50.86) in other race athletes. Mean ADI was higher in black versus white athletes (mean difference 17.68, 95% CI 10.25, 25.12; p=0.0036) and black versus other race athletes (mean difference 17.11, 95% CI 4.74, 29.47; p<0.0001). CONCLUSIONS: Black athletes with SCA come from areas with higher neighbourhood socioeconomic deprivation than white or other race athletes with SCA. Our findings suggest that socioeconomic deprivation may be associated with racialised disparities in athletes with SCA.

Atypical Pattern of Leukocytoclastic Vasculitis in Granulomatosis With Polyangiitis.

Granulomatosis with polyangiitis (GPA), formerly Wegener's granulomatosis, is a small- and medium-vessel vasculitis with characteristic cutaneous morphologic presentation and systemic involvement. Most patients have palpable purpura at some point in their disease course, but this is not always the presenting manifestation. This autoimmune disorder can affect a range of organs, with the upper and lower respiratory tract, kidneys, and nervous system being commonly implicated, while gastrointestinal and cardiac involvement is less frequent. This is a 44-year-old female presenting to the emergency department (ED) with polyarthralgia and palpable purpura. Palpable purpura was distributed on the oral palate, elbow, and lower back, and a punch biopsy revealed leukocytoclastic vasculitis (LCV). While this was an atypical distribution for leukocytoclastic vasculitis, the skin biopsy provided the necessary evidence to diagnose GPA. This case characterizes non-specific and atypical signs and symptoms of GPA that all providers should be aware of in order to diagnose the condition early in its disease course.

A case of trichodysplasia spinulosa related to ruxolitinib treated successfully with oral acitretin.

Trichodysplasia spinulosa (TS) is a rare disease that affects immunocompromised patients, characterized by hair-like growths caused by TS-associated polyomavirus infection. Little is known about specific immunosuppressive drugs that can precipitate the condition. We report a case of TS presenting after initiating the oral Janus-associated kinase inhibitor (JAKi) ruxolitinib. A 67-year-old female with a history of allogeneic bone marrow transplant requiring immunosuppression with tacrolimus, prednisone and, more recently, ruxolitinib 5 mg twice daily due to Graft versus Host Disease presented to the clinic with a facial rash. The clinical and histopathological findings in the setting of immunosuppression were consistent with TS. Initial treatments were ineffective, but oral acitretin showed significant improvement after 3 months. Due to the close temporal relationship between the initiation of ruxolitinib and the development of TS, this case suggests that JAKis may contribute to TS development by suppressing the JAK-signal transducer and activator of the transcription pathway's antiviral functions.

Community participation and stakeholder engagement in determining health service coverage: A systematic review and framework synthesis to assess effectiveness.

Background: Community and stakeholder involvement in decision-making to determine publicly-funded health services and interventions is advocated to fulfil citizens' rights and improve health outcomes. The inclusion of public actors, particularly disadvantaged populations, in priority setting for universal health coverage (UHC) is also enshrined in guidance from the World Health Organization (WHO). However, challenges remain in operationalising this policy aim and ensuring that these approaches are effective and equitable. This study aimed to synthesise published evidence on the role of community and stakeholder participation in determining health service coverage. Methods: A systematic review was conducted, searching the Web of Science, Ovid Global Health, and PubMed Central databases from 2000 onwards, including all study types. A framework synthesis approach was used for charting and synthesising data on mechanisms, effectiveness (equity, depth, and stage), and barriers and facilitators for engagement. Results: Twenty-seven relevant studies were identified that involve community actors and other stakeholders in priority setting and decision-making processes for defining health benefit packages and UHC, health technology assessment, and pharmaceutical coverage. Mechanisms of engagement include a wide variety of consultation approaches; participation in decision-making committees, advisory councils, and local planning meetings; and appeals mechanisms. Participation occurs primarily at Data and Dialogue stages of decision-making processes, and we found limited depth of engagement among identified cases. Limited consideration of equity was observed in planning and reporting on community involvement in priority setting. A number of challenges are identified in the literature, which we typologise as institutional, procedural, technical, and structural / normative barriers to meaningful participation. Conclusions: This systematic review identifies key gaps and opportunities in the literature and practice related to effective and equitable community and stakeholder participation in determining health service coverage. It offers essential considerations for planning and executing inclusive approaches to priority setting for publicly-funded health services and interventions and defining health benefit packages for UHC.

Pediatric Education Discharge Support Strategies for Newly Diagnosed Children With Cancer.

BACKGROUND: Discharge education practices vary among institutions and lack a standardized approach for newly diagnosed pediatric oncology patients and their parents. OBJECTIVE: The purpose of this American Nurses Credentialing Center-supported pediatric multisite trial was to determine the feasibility and effectiveness of 2 nurse-led Parent Education Discharge Support Strategies (PEDSS) for families with a child who is newly diagnosed with cancer. INTERVENTIONS/METHODS: A cluster randomized clinical trial design assigned 16 Magnet-designated sites to a symptom management PEDSS intervention or parent support and coping PEDSS intervention. Outcome measures evaluated at baseline, 1, and 2 months after diagnosis include symptom experiences, parent perceptions of care, unplanned service utilization, and parent evaluation of the PEDSS interventions. RESULTS: There were 283 newly diagnosed children and their parent participating in this study. Linear mixed models revealed pain differed over time by the intervention; children in the symptom management group had a greater decrease in pain. Greater nausea and appetite disturbances were experienced by older children in both groups. Fatigue and sleep disturbance showed a significant decrease over time in both groups. The symptom management group reported significantly greater satisfaction with the PEDSS intervention. CONCLUSIONS: This study is among the first to examine the effects of 2 different early-discharge planning strategies for families of a newly diagnosed child with cancer. The evidence supports a standardized discharge education strategy that can be successfully implemented across institutions. IMPLICATIONS FOR PRACTICE: Nurses play a major role in the educational preparation and discharge of newly diagnosed pediatric cancer patients and their families.

Implementing a pediatric oncology nursing multisite trial.

PURPOSE/BACKGROUND: The Parent Educational Discharge Support Strategies (PEDSS) nursing study includes 16 magnet pediatric oncology institutions across the United States and one in Saudi Arabia, evaluating a nurse-led parent educational discharge support strategy for families experiencing a child newly diagnosed with cancer. METHODS: During the first 3 months of the study, a research implementation survey was administered electronically to each site principal investigator to evaluate facilitators and barriers in the research process for this multisite nurse-led pediatric oncology study. RESULTS: Facilitators included nursing leadership support and commitment from the nursing staff. Common barriers reported were the Institutional Review Board process, the consent process, the timing of the intervention, data collection, as well as nursing time for the study. Results from the survey suggest nurse-led research teams were motivated and felt the intervention was easy to deliver. PRACTICE IMPLICATIONS: Nursing practice is enhanced when nurses participate in research and generate evidence regarding best practices within pediatric oncology nursing care. CONCLUSION: Nursing research endeavors focusing on collaborative approaches for implementation can lead to successful nursing studies with careful planning, training and administrative support.

Promoting Direct Care Nurse Engagement in Research in Magnet Hospitals: The Parent Education Discharge Support Strategies Experience.

Multisite study participation provides an opportunity for hospitals to gain access to the resources required to facilitate nursing research. The nurse-led Parent Educational Discharge Support Strategies for children newly diagnosed with cancer (PEDSS) multisite study engaged direct care nurses for successful implementation across 16 Magnet-designated hospitals. This article addresses strategies to overcome barriers to nursing research demonstrated through the PEDSS experience.

Evaluation of the Effectiveness and Tolerability of Mycophenolate Mofetil and Mycophenolic Acid for the Treatment of Morphea.

Importance: First-line systemic therapy for morphea includes methotrexate with or without systemic corticosteroids. When this regimen is ineffective, not tolerated, or contraindicated, a trial of mycophenolate mofetil (MMF) or mycophenolic acid (MPA)-referred to herein as mycophenolate-is recommended; however, evidence to support this recommendation remains weak. Objective: To evaluate the effectiveness and tolerability of mycophenolate for the treatment of morphea. Design, Setting, and Participants: A retrospective cohort study was conducted from January 1, 1999, to December 31, 2018, among 77 patients with morphea from 8 institutions who were treated with mycophenolate. Main Outcomes and Measures: The primary outcome was morphea disease activity, severity, and response at 0, 3 to 6, and 9 to 12 months of mycophenolate treatment. A secondary outcome was whether mycophenolate was a well-tolerated treatment of morphea. Results: There were 61 female patients (79%) and 16 male patients (21%) in the study, with a median age at disease onset of 36 years (interquartile range, 16-53 years) and median diagnostic delay of 8 months (interquartile range, 4-14 months). Generalized morphea (37 [48%]), pansclerotic morphea (12 [16%]), and linear morphea of the trunk and/or extremities (9 [12%]) were the most common subtypes of morphea identified. Forty-one patients (53%) had an associated functional impairment, and 49 patients (64%) had severe disease. Twelve patients received initial treatment with mycophenolate as monotherapy or combination therapy and 65 patients received mycophenolate after prior treatment was ineffective (50 of 65 [77%]) or poorly tolerated (21 of 65 [32%]). Treatments prior to mycophenolate included methotrexate (48 of 65 [74%]), systemic corticosteroids (42 of 65 [65%]), hydroxychloroquine (20 of 65 [31%]), and/or phototherapy (14 of 65 [22%]). After 3 to 6 months of mycophenolate treatment, 66 of 73 patients had stable (n = 22) or improved (n = 44) disease. After 9 to 12 months of treatment, 47 of 54 patients had stable (n = 14) or improved (n = 33) disease. Twenty-seven patients (35%) achieved disease remission at completion of the study. Treatments received in conjunction with mycophenolate were frequent. Mycophenolate was well tolerated. Gastrointestinal adverse effects were the most common (24 [31%]); cytopenia (3 [4%]) and infection (2 [3%]) occurred less frequently. Conclusions and Relevance: This study suggests that mycophenolate is a well-tolerated and beneficial treatment of recalcitrant, severe morphea.

Eosinophilic fasciitis: Current concepts.

Eosinophilic fasciitis (EF) is an uncommon connective tissue disease characterized by abrupt onset of edema, followed by progressive induration of primarily the distal extremities. Patients may exhibit inflammatory arthritis, joint contractures, decreased mobility, and nerve entrapment. Almost half of patients with EF may have coexisting morphea plaques. Classic laboratory studies display peripheral eosinophilia, hypergammaglobulinemia, and elevated inflammatory markers. EF is included in the spectrum of scleroderma like disorders and may be difficult to distinguish from other sclerosing skin disorders. Full-thickness biopsy containing muscle and fascia is considered the gold standard for diagnosis and reveals sclerosis of the middeep dermis, subcutaneous fat, and thickening of the fascia. Magnetic resonance imaging (MRI) has been increasingly utilized to augment diagnostic capabilities. Ultimately, the diagnosis of EF relies upon the combination of characteristic clinical, laboratory, imaging, and histologic findings. Although some patients experience spontaneous remission, systemic corticosteroids (SCS) are the mainstay of treatment. Patients who fail to improve with SCS alone require the addition of a second immunosuppressive drug. Additionally, although data are limited, there is evidence to suggest that initial combination therapy with SCS and methotrexate (MTX) may be most beneficial.

Child Marriage Laws around the World: Minimum Marriage Age, Legal Exceptions, and Gender Disparities.

The marriage of children below 18 is widely recognized in international human rights agreements as a discriminatory global practice that hinders the development and well-being of hundreds of millions of girls. Using a new global policy database, we analyze national legislation regarding minimum marriage age, exceptions permitting marriage at earlier ages, and gender disparities in laws. While our longitudinal data indicate improvements in frequencies of countries with legal provisions that prohibit marriage below the age of 18, important gaps remain in eliminating legal exceptions and gender discrimination.

Teaching the basics: core competencies in global health.

Compelling moral, ethical, professional, pedagogical, and economic imperatives support the integration of global health topics within medical school curriculum. Although the process of integrating global health into medical education is well underway at some medical schools, there remain substantial challenges to initiating global health training in others. As global health is a new field, faculties and schools may benefit from resources and guidance to develop global health modules and teaching materials. This article describes the Core Competencies project undertaken by the Global Health Education Consortium and the Association of Faculties of Medicine of Canada's Global Health Resource Group.