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Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome is a severe adverse drug-induced hypersensitivity reaction. We report a case of DRESS syndrome in a 17-year-old female caused by itraconazole, confirmed by patch testing, that required treatment with both corticotherapy and cyclosporine. Our case highlights the importance of clinical suspicion of this syndrome in pediatric age and the novelty of an antifungal drug being identified as the culprit. [ABSTRACT FROM AUTHOR]

Background: Chronic urticaria is defined as the appearance of urticarial lesions and/or angioedema during a period of more than six weeks. We aimed at developing the Portuguese version of the Chronic Urticaria Quality of Life Questionnaire (CU-Q2oL) and at testing its reliability and the content, construct and criterion validity.Methods: The forward-backward approach to a linguistic equivalence was followed, after which a clinical review and a cognitive debriefing with patients were performed. The intraclass correlation coefficient checked test-retest reliability with patients filling the same questionnaire with one week apart and the Cronbach's alpha indicator assessed the internal consistency. Construct validity was tested by an exploratory factor analysis and by hypothesis tests involving sociodemographic and clinical patient characteristics, including the urticaria control test (UCT). On the other hand, criterion validity was tested through correlations with the Short-Form Health Survey SF-36, EQ-5D-5 L, and the Dermatology Quality of Life Index (DLQI).Results: A total of 162 patients from seven hospital units were included. The mean (standard deviation) age was 42.6 (13.3) and 81.6% were female. CU-Q2oL was entirely filled by all respondents. Internal consistency was 0.947 for the overall score, ranging from 0.661 (limits) to 0.899 (sleep problems) and the corresponding reproducibility indicator was 0.910, based on 23 patients and ranging from 0.711 (swelling) and 0.957 (looks). Exploratory factor analysis in general confirmed the original structure originally obtained by the authors. All CU-Q2oL dimensions were highly correlated with DLQI Index and differentiated well between males and females, and between different levels of wheals and pruritus. In addition, moderate negative correlations were found between Cu-Q2oL scores and the dimensions from SF-36 and EQ-5D-5 L.Conclusions: The satisfactory metric properties confirmed the cultural adaptation and validity of CU-Q2oL into Portuguese population, providing the clinicians with a valid tool to evaluate the impact of chronic urticaria on patient's QoL and therefore adjust their treatment.Trial Registration Number: Not applicable. [ABSTRACT FROM AUTHOR]

We here report the use of benralizumab in a patient with severe PDGFRA-negative idiopathic HES involving three organs and refractory to glucocorticoid and other immunosuppressor therapies. Introduction Hypereosinophilic syndrome (HES) is a rare, heterogeneous group of disorders characterized by persistent, marked blood eosinophilia associated with eosinophil-induced tissue/organ damage, causing a wide range of clinical manifestations [[1]]. In a phase 2 trial with twenty PDGFRA-negative HES patients, benralizumab reduced blood, bone marrow, and tissue eosinophilia, and allowed tapering of immunosuppressive therapies [[2]]. [Extracted from the article]

The Portuguese Severe Asthma Registry (Registo de Asma Grave Portugal, RAG) was developed by an open collaborative network of asthma specialists. RAG collects data from adults and pediatric severe asthma patients that despite treatment optimization and adequate management of comorbidities require step 4/5 treatment according to GINA recommendations. In this paper, we describe the development and implementation of RAG, its features, and data sharing policies. The contents and structure of RAG were defined in a multistep consensus process. A pilot version was pretested and iteratively improved. The selection of data elements for RAG considered other severe asthma registries, aiming at characterizing the patient’s clinical status whilst avoiding overloading the standard workflow of the clinical appointment. Features of RAG include automatic assessment of eligibility, easy data input, and exportable data in natural language that can be pasted directly in patients’ electronic health record and security features to enable data sharing (among researchers and with other international databases) without compromising patients’ confidentiality. RAG is a national web-based disease registry of severe asthma patients, available at asmagrave.pt. It allows prospective clinical data collection, promotes standardized care and collaborative clinical research, and may contribute to inform evidence-based healthcare policies for severe asthma. [ABSTRACT FROM AUTHOR]

Introduction Fungal spores constitute an important fraction of bioaerosols in the atmosphere. Objectives To analyse the content of Alternaria and Cladosporium spores in the atmosphere of Beja and the effect of meteorological conditions on their concentrations. Methodology The daily and hourly data of Alternaria and Cladosporium fungal spores concentration in the atmosphere of Beja were monitored from April 12, 2012 to July 30, 2014, based on the Portuguese Aerobiology Network methodology. The influence of meteorological conditions on the studied types of fungal spore concentrations was assessed through Spearman’s correlation analysis. Results During the study period, 20,741 Alternaria spores and 320,862 Cladosporium spores were counted. In 2013, there were 5,822 Alternaria spores and 123,864 Cladosporium spores. The absolute maximum concentrations of Alternaria and Cladosporium spores were recorded on November 8, 2013, with 211 and 1301 spores/m3, respectively. Temperature, insolation and wind direction parameters showed a positive correlation with Alternaria and Cladosporium spore levels, while relative humidity and precipitation presented a negative correlation, which is statistically significant. Wind speed only showed a statistically significant positive correlation in terms of Alternaria spore levels. Conclusion Alternaria and Cladosporium spores are present in the atmospheric air of Beja throughout the year, with the highest concentration period occurring during spring and autumn. There was a clear effect of meteorological parameters on airborne concentrations of these fungal spores. [ABSTRACT FROM AUTHOR]

Background: Flavonoids exert anti-inflammatory properties and modulate oxidative stress in vitro, suggesting a protective effect on lung function, but epidemiological studies examining this association are scarce. Methods: A stratified random sample was drawn from the GA2LEN screening survey, in which 55,000 adults aged 15 to 75 answered a questionnaire on respiratory symptoms. Post-bronchodilator spirometry was obtained from 2850 subjects. Forced vital capacity (FVC), the ratio between the forced exhaled volume in 1 second (FEV1) and FVC (FEV1/FVC), FVC below lower limit of normal (FVC < LLN), and FEV1/FVC < LLN were calculated. Intake of the six main subclasses of flavonoids was estimated using the GA2LEN Food Frequency Questionnaire. Adjusted associations between outcomes and each subclass of flavonoids were examined with multivariate regressions. Simes' procedure was used to test for multiple comparisons. Results: A total of 2599 subjects had valid lung function and dietary data. A lower prevalence of FVC < LLN (airway restriction) was observed in those with higher total flavonoid (adjusted odds ratio (aOR), higher vs. lowest quintile intake 0.58; 95% Confidence Interval (CI) 0.36, 0.94), and pro-anthocyanidin intakes (aOR 0.47; 95% CI 0.27, 0.81). A higher FEV1/FVC was associated with higher intakes of total flavonoids and pro-anthocyanidins (adjusted correlation coefficient (a β-coeff 0.33; 0.10, 0.57 and a β-coeff 0.44; 95% CI 0.19, 0.69, respectively). After Simes' procedure, the statistical significance of each of these associations was attenuated but remained below 0.05, with the exception of total flavonoids and airway restriction. Conclusions: This population-based study in European adults provides cross-sectional evidence of a positive association of total flavonoid intake and pro-anthocyanidins and ventilatory function, and a negative association with spirometric restriction in European adults. [ABSTRACT FROM AUTHOR]

Beer is one of the most consumed alcoholic beverages worldwide but allergic reactions to this beverage are uncommon. The authors present a case report of a 32-year-old male patient, sent to our Allergy and Immunology Department due to anaphylaxis minutes after Franziskaner beer ingestion. He tolerates all other alcoholic beverages. Prick tests to cereals were positive to wheat, corn and barley, as well as to peach. Prick-to-prick tests were performed with nine beer brands, all positive. Immunoglobulin (Ig)E to Pru p 3 was 14.8 kU/L. Sodium dodecyl sulfate polyacrylamide gel electrophoresis inhibition immunoblotting was performed with the Franziskaner beer extract in solid phase and both cereal extracts (wheat, barley and corn) and Pru p 3 as inhibitors. Extracts from wheat, barley and corn, and Pru p 3 purified protein were able to inhibit almost totally the IgE-binding to the Franziskaner beer extract. It seemed likely that the IgE-binding bands detected in the Franziskaner beer extract could be an LTP from cereals. [ABSTRACT FROM AUTHOR]

Keywords: allergic contact dermatitis; case report; contact urticaria; occupational; patch test; rubber chemicals EN allergic contact dermatitis case report contact urticaria occupational patch test rubber chemicals 471 472 2 09/21/21 20211001 NES 211001 Aquagenic urticaria (AU) is a chronic, rare type of physical urticaria that is elicited by skin contact with water, including sweat and tears.1 Due to its rarity and the existence of other skin diseases caused by water exposure, exclusion of differential diagnoses is essential.2 We, here, describe a patient with concomitant AU and allergic contact dermatitis (ACD) that impaired her work ability during the coronavirus disease 2019 (COVID-19) pandemic. Concomitant allergic contact dermatitis and aquagenic urticaria caused by personal protective equipment in a healthcare worker during the COVID-19 pandemic To exclude other chronic urticarias, we tested for cold urticaria, performed a methacholine skin challenge (intradermal, at concentrations of 0.001 mg/mL, 0.01 mg/mL, and 0.1 mg/mL) and an autologous serum test, all of which were negative. [Extracted from the article]

Three days later, erythematous plaques around each injection site enlarged, diffuse abdominal erythema developed, and the patient reported generalized pruritus (Figure 1). Adult-onset Still's disease, anakinra, case report, delayed hypersensitivity reactions Keywords: adult-onset Still's disease; anakinra; case report; delayed hypersensitivity reactions EN adult-onset Still's disease anakinra case report delayed hypersensitivity reactions 381 382 2 08/16/21 20210901 NES 210901 Anakinra is a human IL-1 receptor antagonist approved for the treatment of adult-onset Still's Disease (AOSD).1,2 Because of its short half-life, anakinra needs to be administered as a daily subcutaneous (SC) injection. [Extracted from the article]

Keywords: allergic contact dermatitis, case report, metals, occupational, patch test EN allergic contact dermatitis, case report, metals, occupational, patch test 523 524 2 11/26/20 20201201 NES 201201 Due to long hours of practice, professional musicians often develop occupational skin diseases.1 String players have the highest risk of contact dermatitis, often suffering from "fiddler's neck",2 most frequently due to irritant contact dermatitis (ICD) while allergic contact dermatitis (ACD) is rarely reported.1 Nickel sulfate and colophonium are the most commonly reported sensitizers. Fiddler's neck: Chin rest-associated irritant contact dermatitis and allergic contact dermatitis in a violin player. [Extracted from the article]

Background: Chronic urticaria (CU) is defined as recurrent urticaria lasting for more than 6 weeks. Objectives: We aimed to characterize the phenotypes of patients with CU refractory to standard dose anti-H1 antihistamine treatment and search for clinical predictors of poor disease control. Methods: Retrospective collection of data regarding clinical characteristics, comorbidities, treatment, and disease control of all adult refractory CU patients presenting to the Allergy and Immunology Department during 1 year. Results: Sixty-one adult patients were included, 74% females, average age 44.5 years (18 to 84 years old). Most patients (78.7%) had initiated CU less than 1 year before enrolment. Chronic spontaneous urticaria (CSU) accounted for 55.7% of the patients, CSU associated with chronic inducible urticaria (CIndU) as a comorbidity for 44.3%, and angioedema was present in 55.7%. Medically-confirmed psychiatric disorders were present in 78.7%. Complementary diagnostic tests were performed in cases with more severe presentation (UAS7 ≥ 28 and/or UCT < 12) or with longer evolution (> 1 year), corresponding to 42 tested patient. Evidence for autoimmunity (positive anti-thyroid peroxidase antibodies, anti-nuclear antibodies or autologous serum test) was found in 45.2% (n = 19/42), and high C-reactive protein was present in 14.3% (n = 6/42), half of these also had positive antinuclear antibodies. Forty-six patients (75.4%) had at least one significant exacerbation, requiring medical appointment, emergency room, hospitalization or job absenteeism. The number of exacerbations correlated with the presence of angioedema (p = 0.022), with a recent diagnosis (< 1 year), and with higher UAS7 severity (p = 0.006). Although ClndU was associated with poor symptom control (p = 0.022), it was also associated with less exacerbations requiring medical observation or hospitalization (p = 0.015). All patients were using antihistamines and 21.3% (n = 13) of them were also under treatment with omalizumab, ciclosporine or montelukast for disease control. Conclusions: Autoimmunity can affect about half of the patients with severe or long-term CU. UAS7 and angioedema are associated with disease exacerbations. UAS7 and UCT presented unequal accuracy, with UAS7 better associating with the occurrence of exacerbations and treatment doses. Patients with refractory CU frequently present psychiatric disorders. Accurate diagnostic tests, namely autoimmune parameters and inflammatory markers, should be recommended in some individual cases. [ABSTRACT FROM AUTHOR]

Allergic diseases are a major health concern worldwide. Pollens are important triggers for allergic rhinitis, conjunctivitis and asthma. Proteases released upon pollen grain hydration appear to play a major role in the typical immunological and inflammatory responses that occur in patients with allergic disorders. In this study, we aimed to identify specific proteolytic activity in a set of pollens with diverse allergenic potential. Diffusates from Chenopodium album, Plantago lanceolata and Eucalyptus globulus were added to a confluent monolayer of Calu-3 cells grown in an air-liquid interface system. We identified serine proteases and metalloproteinases in all pollen diffusates investigated. Proteases found in these pollen diffusates were shown to compromise the integrity of the lung epithelial barrier by disrupting transmembrane adhesion proteins E-cadherin, claudin-1 and Occludin, as well as, the cytosolic complex zonula occludens-1 (ZO-1) resulting in a time-dependent increase in transepithelial permeability. Tight junction disruption and increased transepithelial permeability facilitates allergen exposure to epithelial sub-layers contributing to the sensitization to a wide range of allergens. These pollen extracts also induced an increase in the release of interleukin 6 (IL-6) and interleukin 8 (IL-8) cytokines measured by flow cytometry possibly as a result of the activation of protease-activated receptors 2 (PAR-2). [ABSTRACT FROM AUTHOR]

The prevalence of food allergy (FA) has increased too rapidly, possibly due to environmental factors. The guidelines recommend strict allergen avoidance, but FA is still the main cause of anaphylaxis in all age groups. Immunotherapy is the only treatment able to change the course of allergic disease, and oral immunotherapy (OIT) is the more effective route in FA. However, it carries the risk of adverse reactions, including anaphylaxis. To improve OIT safety, adjuvant therapy with the immunoglobulin E (IgE) monoclonal antibody omalizumab has been extensively used. Results suggest particular benefit in patients with high risk of fatal anaphylaxis. An alternative approach is to use omalizumab instead of OIT to prevent severe allergic reactions upon accidental exposure. This paper reviews current evidence regarding IgE‐mediated FA, focusing on natural tolerance and food sensitization acquisition, and on avoidance measures and their limitations. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 79-year-old male with a past history of tophaceous gout, hypertension and heart failure developed generalized pruritic exanthema10 days after he started taking amlodipine and allopurinol. Topics include spironolactone is an aldosterone antagonist used as a diuretic and antihypertensive drugtients.