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Leiomyomas are benign tissue neoplasms that arise from smooth muscles. They are divided into three types according to muscle origin, namely, piloleiomyoma, angioleiomyoma and genital leiomyoma. Piloleiomyoma is a type of cutaneous leiomyoma arising from arrectores pilorum. They are characterized by firm, round to oval, skin coloured to brownish lesions that may present as either a solitary nodule or multiple papulonodules. They may be asymptomatic or painful at presentation and the pain can be triggered by cold exposure or light touch. It is often localized on the trunk and extremities, but less than 1% of leiomyomas occur in the head and neck area. Here we report a case of piloleiomyoma which located on the face in the form of tumoral lesions. We recommend that leiomyomas must be kept in mind in the clinical differantial diagnosis of painful papulonodules. [ABSTRACT FROM AUTHOR]

The article discusses the case of a 15-year-old annular lichenoid dermatitis patient who had treated with topical tacrolimus as a treatment option due to the side effects of long-term potent steroid use. It mentions the consideration of the differential diagnosis of annular plaques of young patients as the treatment option of the disease. It offers the history of the cases reported of the disease.

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Background Method Results Conclusion CRUSE® is an app that allows patients with chronic spontaneous urticaria (CSU) to monitor their daily disease activity through the use of visual analogue scales (VASs). We aimed to determine the concurrent validity, reliability, responsiveness and minimal important difference (MID) of CRUSE® VASs.We evaluated the properties of three daily VASs: VAS for how much patients were affected by their CSU (‘VAS urticaria’), VAS for the impact of urticaria on work/school productivity (‘VAS productivity’) and the VAS of EQ‐5D. Concurrent validity was assessed by measuring the association between each VAS and the Urticaria Activity Score (UAS). Intra‐rater reliability was determined based on the data of users providing multiple daily questionnaires within the same day. Test–retest reliability and responsiveness (ability to change), respectively, were tested in clinically stable and clinically unstable users. MIDs were determined using distribution‐based methods.We included 5938 patients (67,380 days). Concurrent validity was high, with VAS urticaria being more strongly associated with the UAS score than the remaining VASs. Intra‐rater reliability was also high, with intraclass correlation coefficients (ICC) being above 0.950 for all VASs. Moderate‐high test–retest reliability and responsiveness were observed, with reliability ICC being highest for VAS EQ‐5D and responsiveness being highest for VAS urticaria. The MID for VAS urticaria was 17 (out of 100) units, compared to 15 units for VAS productivity and 11 units for VAS EQ‐5D.Daily VASs for CSU available in the CRUSE® app display high concurrent validity and intra‐rater reliability and moderate‐high test–retest reliability and responsiveness. [ABSTRACT FROM AUTHOR]

The article in Clinical & Experimental Allergy explores the connection between lymphopenia, basopenia, and eosinopenia in chronic spontaneous urticaria (CSU) patients, particularly those with autoimmune CSU. The study involved 300 CSU patients and found that lymphopenia was associated with lower counts of basophils, eosinophils, monocytes, and platelets. The research suggests that lymphopenia may reflect the migration of lymphocytes from the bloodstream into skin lesions, similar to basophils and eosinophils. The study also identified associations between basopenia and high anti-C1q levels, supporting the hypothesis that these antibodies may be a marker of autoimmune CSU. [Extracted from the article]

This article discusses a study that aimed to identify potential biomarkers for chronic spontaneous urticaria (CSU), a skin disease characterized by severe itching. The researchers collected blood samples from 118 CSU patients and analyzed the plasma proteomics to identify associations with key disease characteristics. They found that certain plasma proteins were significantly associated with clinical traits such as sex, positive basophil histamine release assay (BHRA), angioedema, and concomitant chronic inducible urticaria (CIndU). However, these proteins were not associated with the response to omalizumab treatment, which is commonly used as a second-line treatment for CSU. The findings provide insights into the molecular mechanisms underlying CSU and may contribute to personalized management of the disease. The document is a list of acknowledgements and conflict of interest statements for the study, with no conflicts of interest reported by the authors. Two graphs labeled "Data S1" and "Data S2" are included. [Extracted from the article]

Annular urticarial configurations are often associated with acute and chronic urticaria. Such lesions may be short-lived, migratory, transient, pruritic, and resolving with no residual evidence, making the diagnosis of urticaria an obvious one. Annular urticarial lesions can be the presenting signs of various cutaneous and systemic diseases. The differentiation of urticarial lesions may be made by considering the duration of an individual lesion longer than 24 hours, with burning and pain sensation in the lesions or lack of pruritus; skin marks such as postinflammatory pigmentation or purpura after resolution of the lesions; associated scaling or vehiculation in the lesions; systemic symptoms such as arthralgia, fever or fatigue; and several abnormal laboratory findings. The main differential diagnoses of annular urticarial lesions include urticarial vasculitis, autoinflammatory syndromes, hypersensitivity reactions, and connective tissue diseases. [ABSTRACT FROM AUTHOR]

A 58-year-old, gravida 6, obese woman presented with a pruritic yellowish plaque around the umbilicus, which first appeared about 3 years ago. She also had flat yellow papules on the axilla and neck. After a burn from a heating device, a few perforating papules and a violaceous hue occurred on the plaque. Histological examination revealed pathological elastic fibers with a keratotic plug and perforation in the deep dermis, which was consistent with perforating pseudoxanthoma elasticum. This case indicates that perforating pseudoxanthoma elasticum, a variant of hereditary pseudoxanthoma elasticum may perforate due to mechanical factors. [ABSTRACT FROM AUTHOR]

Chronic urticaria is a common and debilitating mast cell-driven skin disease presenting with itchy wheals, angio-oedema, or both. Chronic urticaria is classified as spontaneous (without definite triggers) and inducible (with definite and subtype-specific triggers; eg, cold or pressure). Current management guidelines recommend step-up administration of second-generation H1-antihistamines to four-fold the approved dose, followed by omalizumab and ciclosporin. However, in many patients, chronic urticaria does not respond to this linear approach due to heterogeneous underlying mechanisms. A personalised endotype-based approach is emerging based on the identification of autoantibodies and other drivers of urticaria pathogenesis. Over the past decade, clinical trials have presented promising options for targeted treatment of chronic urticaria with the potential for disease modification, including Bruton's tyrosine kinase inhibitors, anti-cytokine therapies, and mast cell depletion. This Therapeutics article focuses on the evidence for these novel drugs and their role in addressing an unmet need for personalised management of patients with chronic urticaria. [ABSTRACT FROM AUTHOR]

Introduction: Chronic inducible urticaria (CIndU) is a subtype of chronic urticaria (CU), which requires specific triggers to occur. Despite their common occurrence, treatment response rates and predictors of treatment responses are largely lacking in the literature. This study evaluates antihistamine (AH) and omalizumab response rates in the most common CIndU subtypes and examines whether certain features can predict treatment responses. Methods: We retrospectively analyzed CU patients with at least one CIndU subtype and performed comparisons between subgroups, in a total of 423 patients (70% CIndU, 30% chronic spontaneous urticaria [CSU] plus CIndU). Results: The treatment response rates in CIndU were 51.6%, 51.5%, and 86.5% with standard-dose second-generation H1-antihistamines (sgAHs), updosed/combined sgAH, and omalizumab, respectively. Overall AH response was higher in CIndU than CSU plus CIndU (78.3% vs. 62%, p = 0.002) and in symptomatic dermographism (SD) and cold urticaria (ColdU) than cholinergic urticaria (ChoU) (83.2% vs. 78.3 vs. 60.9%, p = 0.04). AH-refractory patients had a longer disease duration (45.2 ± 56.7 months vs. 37 ± 51.9 months, p = 0.04), more angioedema, accompanying CSU, mixed CIndU subtypes (37.5% vs. 21.1%, p = 0.003; 45.1% vs. 27.1%, p = 0.002; 8.8% vs. 2.4%, p = 0.014), and lower baseline urticaria control test scores (5.86 ± 3.3 vs. 8.6 ± 3.6, p < 0.001) than AH-responsive patients. Conclusion: CIndU exhibits a good response to both AHs and omalizumab. Notably, the response to AHs is more pronounced in SD and ColdU compared to ChoU. Disease duration, angioedema, accompanying CSU, mixed CIndU, and lower baseline UCT scores may be used to predict AH treatment outcome in CIndU. [ABSTRACT FROM AUTHOR]

Chronic urticaria is a disease characterized by the appearance of weals, angioedema or both longer than 6 weeks. Degranulation of cutaneous or submucosal mast cells leads to release of mediators including histamine resulting in redness, swelling and itch. Because mast cells are widely distributed throughout the body, the question is why they are not activated systemically or does systemic activation occur without overt end organ dysfunction? We have conducted an exploratory literature search for reports that have evidence of organ-specific dysfunction in chronic urticaria that might justify prospective observational studies. This search revealed some evidence of systemic effects of chronic urticaria in cardiac, respiratory, gastrointestinal, central nervous and musculo-skeletal systems. The relevance of these findings needs to be further determined. However, they justify prospective studies in larger numbers of patients and at different stages of disease activity. [ABSTRACT FROM AUTHOR]

In this study, the European Academy of Dermatology and Venereology (EADV) Task Forces on Quality of Life and Patient‐Oriented Outcomes and Urticaria and Angioedema has examined the Health‐Related Quality of Life (HRQoL) measurement in the treatment of urticaria. The Dermatology Life Quality Index was the most frequently used HRQoL instrument in clinical trials on urticaria. Many reports of clinical trials of urticaria gave no exact numeric results related to HRQoL changes, making clear conclusions and comparisons with other studies impossible. The interpretation of HRQoL impairment data is more difficult when assessed by instruments without severity stratification systems. The minimal clinically significant difference (MCID) is a more clinically oriented and relevant parameter than depending on statistically significant changes in HRQoL scores. Therefore, using HRQoL instruments with established MCID data in clinical trials and clinical practice is preferred. [ABSTRACT FROM AUTHOR]

Background: Chronic spontaneous urticaria (CSU) is unpredictable and can severely impair patients' quality of life. Patients with CSU need a convenient, user‐friendly platform to complete patient‐reported outcome measures (PROMs) on their mobile devices. CRUSE®, the Chronic Urticaria Self Evaluation app, aims to address this unmet need. Methods: CRUSE® was developed by an international steering committee of urticaria specialists. Priorities for the app based on recent findings in CSU were defined to allow patients to track and record their symptoms and medication use over time and send photographs. The CRUSE® app collects patient data such as age, sex, disease onset, triggers, medication, and CSU characteristics that can be sent securely to physicians, providing real‐time insights. Additionally, CRUSE® contains PROMs to assess disease activity and control, which are individualised to patient profiles and clinical manifestations. Results: CRUSE® was launched in Germany in March 2022 and is now available for free in 17 countries. It is adapted to the local language and displays a country‐specific list of available urticaria medications. English and Ukrainian versions are available worldwide. From July 2022 to June 2023, 25,710 observations were documented by 2540 users; 72.7% were females, with a mean age of 39.6 years. At baseline, 93.7% and 51.3% of users had wheals and angioedema, respectively. Second‐generation antihistamines were used in 74.0% of days. Conclusions: The initial data from CRUSE® show the wide use and utility of effectively tracking patients' disease activity and control, paving the way for personalised CSU management. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 36-year-old female patient with a swelling on her right leg which was not followed by any subjective complaints or history of trauma. Histopathological test disclosed a slight acanthosis in the epidermis, a symmetrical lesion comprising of melanocytes, and a slight lymphocytic infiltration around the vessels. It notes that Desmoplastic Spitz Naevus (DSN), a melanocytic lesion, can be mistaken for dermatofibroma due to similar location and clinical image.

Background: Urticarial vasculitis (UV) should be differentiated from chronic spontaneous urticaria (CSU) in patients initially presenting with recurrent wheals, although criteria for differential diagnosis remain ill‐defined. Objectives: To set the goals, define criteria and unmet needs in UV diagnosis and differential diagnosis with CSU, and explore the possibility of coexistence of both diseases. Methods: Thirteen experts experienced in UV research participated in a Delphi survey of European Academy of Allergy and Clinical Immunology taskforce. This Delphi survey involved three rounds of anonymous responses to n = 32 questions with the aim to aggregate the experts' opinions and to achieve consensus. Urticaria specialists (n = 130, most from Urticaria Centers of Reference and Excellence) evaluated the consensus statements and recommendations in the fourth and final round. Results: The panel agreed that essential criteria to guide a skin biopsy in patients with recurrent wheals should include at least one of the following features: wheal duration >24 h, bruising/postinflammatory hyperpigmentation, and systemic symptoms. Leukocytoclasia and fibrin deposits were identified as a minimum set of UV histological criteria. As agreed by the panel members, CSU and normocomplementemic UV (NUV) may coexist in some patients. Conclusions: The use of established criteria for the diagnosis and differential diagnosis of UV in patients with recurrent wheals can help guide the diagnostic approach and prompt earlier treatment. Further studies should investigate whether CSU and NUV are different entities or part of a disease spectrum. [ABSTRACT FROM AUTHOR]

The signs and symptoms of chronic urticaria (CU) are caused by the activation and degranulation of skin mast cells (MCs). Recent studies have added to our understanding of how and why skin MCs are involved and different in CU. Also, novel and relevant mechanisms of MC activation in CU have been identified and characterized. Finally, the use of MC-targeted and MC mediator-specific treatments has helped to better define the role of the skin environment, the contribution of specific MC mediators, and the relevance of MC crosstalk with other cells in the pathogenesis of CU. Here, we review these recent findings and their impact on our understanding of CU, with a focus on chronic spontaneous urticaria (CSU). Also, we highlight open questions, issues of controversy, and unmet needs, and we suggest what studies should be performed moving forward. [ABSTRACT FROM AUTHOR]

Background: Chronic spontaneous urticaria (CSU) is a common disease both in the pediatric and in the adult population. However, there are differences between the two patient populations with respect to etiological factors, comorbidities, and treatment responses. Our aim was to determine differences between pediatric and adult CSU in terms of clinical characteristics, laboratory parameters, comorbidities, response to treatment, and indicators of response. Methods: A retrospective analysis of CSU patients was performed. Data regarding differences between pediatric and adult CSU patients were analyzed. Indicators of treatment response were determined separately in both pediatric and adult patients. Results: Of 751 CSU patients (162 pediatrics and 589 adults), female dominancy (48.8% vs. 69.6%) and rate of angioedema (19.1% vs. 59.8%) were lower, and disease duration (5 months vs. 12 months) was shorter in pediatric patients. Anti‐TPO positivity (24.7% vs. 9%), elevated CRP (46.5% vs. 11.1%), eosinopenia (38.5% vs. 18.1%), and skin prick test positivity (39.3% vs. 28.8%) were significantly more frequent in adult patients. Response to antihistamines was higher in the pediatric group, and only 7% used omalizumab versus 20.8% in the adults. The comparisons were also performed between <12‐year and ≥12‐year patients and yielded similar results. Conclusion: Pediatric CSU shows distinct characteristics such as lower incidence of angioedema and antithyroid antibodies, and it responds better to antihistamines. These suggest that CSU becomes more severe and refractory in adolescents and adults. Adolescent CSU shows features similar to adult CSU rather than pediatric CSU. [ABSTRACT FROM AUTHOR]

Hydroxychloroquine (HCQ) is a synthetic antimalarial drug that has been used widely in the treatment of dermatologic and rheumatologic diseases. Anti-malarials cause many adverse reactions, with ocular, neurological, and cutaneous side effects being the most frequently described. The most common cutaneous adverse reaction is skin pigmentation. Two different clinical patterns in the same patient and erythema multiforme (EM) due to HCQ have rarely bear reported. Here, we report a 51-year-old man with rheumatoid arthritis who developed maculopapular and pustular lesions, followed by EM after HCQ treatment. [ABSTRACT FROM AUTHOR]

Importance: Treating patients with chronic urticaria using omalizumab has been shown to be safe and effective in randomized clinical trials. Multinational studies on long-term omalizumab performance in chronic urticaria in clinical practice settings are lacking, especially on drug survival. Drug survival, which refers to the length of time that patients are treated with a specific drug, is a comprehensive outcome covering effectiveness, safety, and patient and physician preferences. Furthermore, little is known about the reasons and potential predictors for omalizumab discontinuation., Objective: To investigate omalizumab drug survival as well as reasons and potential predictors for discontinuation in a large, diverse population., Design, Setting, and Participants: This international multicenter cohort study was conducted at 14 Urticaria Centers of Reference and Excellence in 10 countries, including all patients with chronic urticaria from these centers who were ever treated with omalizumab., Main Outcomes and Measures: Drug survival analysis was performed to assess time to discontinuation. Patient characteristics and treatment protocols were investigated by Cox regression analysis to identify potential predictors for omalizumab discontinuation., Results: In 2325 patients with chronic urticaria who started omalizumab between June 2009 and July 2022, the mean (SD) age of the cohort was 42 (6) years, and 1650 participants (71%) were female. Overall omalizumab survival rates decreased from 76% to 39% after 1 to 7 years, respectively (median survival time, 3.3 [95 % CI, 2.9-4.0] years), primarily due to discontinuation from well-controlled disease in 576 patients (65%). Ineffectiveness and adverse effects were reasons for discontinuation in a far smaller proportion of patients, totaling 164 patients (18%) and 31 patients (4%), respectively. Fast treatment response was associated with higher rates of omalizumab discontinuation due to well-controlled disease (hazard ratio, 1.45 [95% CI, 1.20-1.75]), and disease duration of more than 2 years was associated with lower rates of discontinuation due to well-controlled disease (HR, 0.81 [95% CI, 0.67-0.98]). Immunosuppressive cotreatment at the start of omalizumab and autoimmune disease was associated with a higher risk for discontinuation due to ineffectiveness (HR, 1.65 [95% CI, 1.12-2.42]). The presence of spontaneous wheals (HR, 0.62 [95% CI, 0.41-0.93]) and access to higher dosages (HR, 0.40 [95% CI, 0.27-0.58) were both associated with a lower risk for discontinuation of omalizumab due to ineffectiveness., Conclusion and Relevance: This multinational omalizumab drug survival cohort study demonstrated that treatment of chronic urticaria with omalizumab in a clinical setting is effective and safe, and well-controlled disease is the main reason for treatment discontinuation. These findings on omalizumab drug survival rates and reasons and potential predictors for discontinuation may guide patients and physicians in clinical decision-making and expectation management. These results may call for the identification of biomarkers for chronic urticaria remission in complete responders to omalizumab treatment.

Introduction: Biomarkers that could reliably anticipate the effectiveness of antihistamines and omalizumab in treating chronic spontaneous urticaria (CSU) have not been conclusively identified. Our objective was to examine how eosinophilic cationic protein (ECP), tryptase, D-dimer, and total Immunoglobulin E (IgE) impact the response to antihistamine and omalizumab treatments in individuals with CSU., Methods: In this cross-sectional retrospective study, CSU patients that had undergone treatment with either antihistamines or omalizumab for a minimum of 12 weeks between 2015 and 2021 at an Allergy and Immunology Department were analyzed. Several demographic and laboratory parameters including eosinophil counts, mean platelet volüme (MPV), sedimentation, C-reactive protein (CRP), antinuclear antibodies (ANA) and Anti-thyroperoxidase (Anti-TPO) and total IgE, tryptase, ECP and D-dimer were retrived from patient files. The association of these biomarkers with Urticaria Control Test (UCT) and the effect of these biomarkers on treatment response were evaluated. Treatment response was assessed using the UCT, with a score of UCT ≥ 12 indicating a responder and UCT < 12 indicating a non responder., Results: The patients in the omalizumab group were older, had a longer disease duration and had worse urticaria control (lower baseline UCT scores). 421 patients were treated with antihistamines and 88 patients were treated with omalizumab. ECP was found to be inversely correlated with baseline UCT ( p  < 0.001 r=-0.268). ECP and D-dimer levels of non-responder patients in the antihistamine group were significantly higher than in responder patients (ECP: 49 ng/mL vs 28.1 ng/mL, p  < 0.001) (D-dimer: 0.60 mg/L vs 0.30 mg/L, p  < 0.001), while there were no significant difference in terms of tryptase and total IgE. These four biomarkers were similar, in omalizumab responders and non responders., Conclusion: In this study with CSU, we looked at predictors of responses to treatments. ECP can serve as a marker of poor urticaria control and may predict antihistamine refractoriness along with D-dimer.

Chronic urticaria (CU) is a common and long-lasting mast cell-mediated skin disease associated with psychiatric and autoimmune comorbidities, high economic costs, and considerable impact on quality of life. Available therapies show limited efficacy in many CU patients, which may be related to distinct underlying pathophysiology. Targeted and disease-modifying treatments with higher and broader efficacy are needed and are under development for CU. These novel drugs, small molecules, and monoclonal antibodies target mast cells and their receptors, signaling pathways, or mediators and other immune cells. In this article, the authors focus on the most promising emerging therapeutics in advanced development and discuss their potential place in future management of CU., Competing Interests: Disclosure MMu: is or recently was a speaker and/or advisor for and/or has received research funding from AstraZeneca, Blueprint, Takeda and Roche. E. Kocatürk is or recently was a speaker and/or advisor for and/or has received research funding from Novartis, Menarini, LaRoche Posey, Sanofi, Bayer, Germany. M. Maurer is or recently was a speaker and/or advisor for and/or has received research funding from Allakos, Allerdia, Amgen, United States, Aralez, Argenx, Netherlands, AstraZeneca, United Kingdom, Celldex, United States, Centogene, CSL Behring, United States, FAES, United States, Genentech, United States, GIInnovation, Innate Pharma, France, Kyowa Kirin, LEO Pharma, Denmark, Lilly, Menarini, Moxie, Novartis, Switzerland, Pfizer, United States, Roche, Switzerland, Sanofi, United States/Regeneron, United States, Third Harmonic Bio, UCB, Belgium, and Uriach. P. Kolkhir was a speaker/consultant and/or advisor for and/or has received research funding from Novartis, ValenzaBio and Roche outside of submitted work., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Chronic spontaneous urticaria (CSU) can present with non-skin related symptoms (NSRS), including recurrent unexplained fever, joint, bone, or muscle pain (JBMP), and malaise, which also occur in other conditions that manifest with wheals (eg, urticarial vasculitis or autoinflammatory disorders) or without wheals (eg, infection)., Objective: We sought to determine the rate of patients with CSU affected by fever, JBMP, and malaise, their trigger factors, links with clinical and laboratory characteristics, and their impact on everyday life and treatment responses., Methods: We analyzed baseline data from the Chronic Urticaria Registry of 2,521 patients with CSU who were aged 16 years or older., Results: One third of CSU patients (31.2%; 786 of 2,521) had one or more NSRS, including recurrent fever (5.3%), JBMP (19.1%), and/or malaise (18.6%). In a multivariable analysis, having one or more of these NSRS correlated with food and infection as trigger factors of urticaria (adjusted odds ratio [aOR] = 1.7 and 1.5), wheals of 24 hours or greater duration (aOR = 2.5), sleep disturbance (aOR = 2.4), anxiety (aOR = 2.8), comorbid atopic dermatitis (aOR = 2.1), gastrointestinal disease (aOR = 1.8), elevated leukocytes (aOR = 1.7) and erythrocyte sedimentation rate (aOR = 1.5). In a bivariate analysis, these NSRS were additionally associated with higher disease activity (weekly Urticaria Activity Score, median: 21 vs 14; P = .009), longer disease duration (years, median: 2 vs 1; P = .001), the presence of angioedema (74.6% vs 58.7%; P < .001), worse quality of life (Chronic Urticaria Quality of Life Questionnaire, median: 42 vs 29; P < .001) and more frequent poor control of CSU (78% vs 69%; P < .001)., Conclusions: The presence of NSRS in a subpopulation of patients with CSU points to the need for better control of the disease, exclusion of comorbid conditions, and/or exclusion of urticarial vasculitis and urticarial autoinflammatory diseases., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Introduction: Urticaria, a mast cell-mediated skin disease, manifests as acute or chronic, with the latter divided into spontaneous and inducible types and requires individualized management, including identifying triggers and comorbidities. Antihistamines, particularly the second generation group, form the mainstay of primary treatment plans consisting of dosage adjustments and/or in combination with other treatment modalities depending on underlying disease control., Areas Covered: A literature search was conducted using 'antihistamines,' 'urticaria,' 'pharmacogenomics,' 'genomics,' 'biomarkers' and 'treatment response' as key words. In this review, we focus on the comprehensive understanding and application of antihistamines in managing adult and adolescent patients with chronic urticaria., Expert Opinion: Using antihistamines to treat urticaria is set to change significantly, focusing more on personalized medicine and identifying key biomarkers to enhance treatment response prediction. These changes aim to make treatments more specific and cost-effective by avoiding unnecessary tests. Applying new approaches in everyday clinical care faces challenges like proving the biomarkers' reliability, updating current guidelines, and incorporating individualized treatments into standard procedures. Efforts should now concentrate on finding easy-to-use biomarkers, improving access to pharmacogenomics, understanding why some patients are resistant to treatment, and creating more specific treatment options based on patient needs.

Background: Determination of control level in recurrent angioedema (RAE) is necessary to guide management. Here, we validated a Turkish version of the angioedema control test (AECT) for 4-week (AECT-4wk) and for 3-month (AECT-3mth) and assessed their utility in monitoring RAE., Method: The recommended structured translation process for patient-reported outcome measures was completed. The final versions were administered to 51 patients with mast cell-mediated angioedema (MMAE) and 38 patients with hereditary angioedema, and the minimal clinically important difference (MCID) was determined. Additionally, anchor surveys comprising angioedema activity score for 28 days (AAS-28 day), visual analog score for angioedema control, Likert scale for the control level from the patient's perspective (LS-AEC), angioedema quality of life, short form-12 (SF-12) and patients' assessment of treatment sufficiency were applied., Results: The Turkish AECT versions showed good convergent validity with a substantial correlation with anchor tools and known-group validity. Excellent internal consistency and reproducibility were observed. Equal or more than 10 of 16 points scored with the AECT-4wk and AECT-3mth identified patients with well-controlled disease. The disease activity, control and burden parameters were consistent with the disease control level defined depending on the cut-off point 10 of AECT. Three-point changes in AECT-4wk and -3 mt could detect MCID in disease control in all patients., Conclusions: Turkish AECT versions are valid and reliable tools for assessing and monitoring disease control in patients with RAE. The use of the Turkish versions of the AECT in routine patient care, clinical trials and angioedema research is recommended., (© 2024 The Authors. Clinical and Translational Allergy published by John Wiley & Sons Ltd on behalf of European Academy of Allergy and Clinical Immunology.)

Background: Although there have been significant advances in the treatment of chronic spontaneous urticaria (CSU) in recent years, there remains a lack of clear guidance on when and how to step down treatment in responders. This study aims to investigate stepping down approaches of different steps of CSU treatment from a global perspective., Methods: "Stepping down chronic spontaneous urticaria treatment" (SDown-CSU) is an international, multicenter, observational, cross-sectional, survey-based study of the Urticaria Centers of Reference and Excellence (UCARE) network. The questionnaire included 48 questions completed by physicians in the UCARE network., Results: Surveys completed by 103 physicians from 81 UCAREs and 34 countries were analyzed. Seventy-eight percent of the participants responded that they had a national urticaria management guideline written by their professional societies and 28% responded that they had to operate under a regulatory guideline proposed by central health funding organizations. Seventy-two and 58.7% of these national recommendations do not contain any detailed information on when and/or how CSU treatment should be discontinued. There was a lack of detailed information on antihistamines and cyclosporine in particular. A predefined maximum duration was generally not applicable to omalizumab and cyclosporine (81% and 82%, respectively). Nearly all UCAREs step down omalizumab within 6 months from the first controlled status and 42% discontinue cyclosporine after 6 months regardless of the control status., Conclusions: The findings from the SDown-CSU study clearly highlight a global need for guidance on the process of stepping down treatment in CSU. Additionally, the study offers a step-down algorithm applicable to all stages of CSU treatment., (© 2024 The Authors. Clinical and Translational Allergy published by John Wiley & Sons Ltd on behalf of European Academy of Allergy and Clinical Immunology.)

Background: Chronic spontaneous urticaria (CSU) is unpredictable and can severely impair patients' quality of life. Patients with CSU need a convenient, user-friendly platform to complete patient-reported outcome measures (PROMs) on their mobile devices. CRUSE ® , the Chronic Urticaria Self Evaluation app, aims to address this unmet need., Methods: CRUSE ® was developed by an international steering committee of urticaria specialists. Priorities for the app based on recent findings in CSU were defined to allow patients to track and record their symptoms and medication use over time and send photographs. The CRUSE ® app collects patient data such as age, sex, disease onset, triggers, medication, and CSU characteristics that can be sent securely to physicians, providing real-time insights. Additionally, CRUSE ® contains PROMs to assess disease activity and control, which are individualised to patient profiles and clinical manifestations., Results: CRUSE ® was launched in Germany in March 2022 and is now available for free in 17 countries. It is adapted to the local language and displays a country-specific list of available urticaria medications. English and Ukrainian versions are available worldwide. From July 2022 to June 2023, 25,710 observations were documented by 2540 users; 72.7% were females, with a mean age of 39.6 years. At baseline, 93.7% and 51.3% of users had wheals and angioedema, respectively. Second-generation antihistamines were used in 74.0% of days., Conclusions: The initial data from CRUSE ® show the wide use and utility of effectively tracking patients' disease activity and control, paving the way for personalised CSU management., (© 2024 The Authors. Clinical and Translational Allergy published by John Wiley & Sons Ltd on behalf of European Academy of Allergy and Clinical Immunology.)

Introduction: The internet is a popular source of health information including images of disease manifestations. Online photographs of skin lesions may aid patients in identifying their disease, if these pictures are of good quality and of the disease they claim to show. If not, patients may be at risk of delayed diagnosis, misdiagnosis, and suboptimal treatment. For urticaria, the mismatch rate and quality of online pictures are unknown. The objective of this study was therefore to evaluate the content and quality of online images of urticaria., Methods: The search term "urticaria" was applied to Google Images and Shutterstock. The top 100 photographs from each search engine were retrieved on October 9th, 2022. Illustrations, drawings, and heavily edited photographs were excluded. Each image was evaluated for patient characteristics, characteristics of urticarial lesions, and image quality., Results: Across 194 unique images of urticaria (after removing duplicates), 35 (18.0%) did not depict urticarial lesions, and 38 (19.6%) were ambiguous. Less than two-thirds of images 121 (62.4%) showed bona fide urticarial lesions. Pictures of urticarial lesions under-represented children and did not reflect female preponderance of the disease. Images predominantly depicted urticaria lesions on Caucasian skin (59.8%) and were typical of spontaneous rather than inducible urticaria. Only 3 (1.5%) pictures showed angioedema, a common clinical sign in patients with urticaria. The overall quality of online urticaria pictures was mostly good or very good., Conclusion: Physicians and patients should be aware that one in five online pictures of urticaria does not show urticarial skin lesions, and children, females, non-Caucasian patients, inducible urticaria, and angioedema are under-represented. These findings should prompt efforts to improve the accuracy and representativeness of online urticaria pictures., (© 2024 S. Karger AG, Basel.)

Background: Chronic urticaria (CU) is characterized by transient wheals and angioedema, which are often not present when patients see their treating physician., Objective: The objective of this study was to evaluate the diagnostic value of smartphone photographs captured by patients prior to their first visit at an urticaria outpatient clinic., Methods: A survey regarding the quality and utility of smartphone photographs of urticarial skin lesions in patients with CU attending the outpatient clinic for the first time was conducted. Up to three random patient-selected photographs of skin lesions were evaluated by a physician., Results: Of 148 patients, 118 (79.7%) had taken photographs of their skin lesions prior to the consultation, and 75% took photographs with the intention of presenting it to their physician. The photographs were of wheals in 90% of the cases and angioedema in 8%. In total, 72% of the smartphone photographs had the skin lesion in focus, 64% had good resolution, and 48% had good lighting. Only 9% of the smartphone photographs were blurred, 10% had bad lighting, 4% had bad resolution, and 8% did not have the lesion in focus. Moreover, 86% of the smartphone photographs were found to be useful for clinical evaluation. At least one photograph of good/very good quality was presented by 86% of the patients, and 97% had at least one photograph that was useful for clinical evaluation., Conclusion: Patients with CU often take smartphone photographs of their skin lesions on their own initiative prior to their first consultation to present the photographs to their physician. These smartphone photographs are very often of good quality and suitable for clinical evaluation., (© 2023 The Author(s). Published by S. Karger AG, Basel.)

Introduction: Health apps play an increasing role in everyday healthcare, especially for chronic diseases. The Chronic Urticaria Self Evaluation (CRUSE) is a new mobile health app for chronic spontaneous urticaria (CSU) patients, which replaces disease tracking via paper and pen, thus making disease monitoring more convenient, increasing tracking compliance, and improving data quality and access. Methods: CRUSE enables patients to complete patient-reported outcome measures on their smartphone and send the results, along with current medication and pictures, to their treating physician via email. CRUSE captures the urticaria (UAS) and angioedema activity (AAS) scores and the urticaria and angioedema control tests (UCT and AECT). In this work, a descriptive analysis of CRUSE users and reported days was performed. The global network of Urticaria Centers of Reference and Excellence (UCARE) provides the app and its data. Results: CRUSE is now available in Germany, Switzerland, Austria, the UK, Italy, Spain, France, and Turkey. Of 620 newly registered users (from July 1 st until November 18 th of 2022), 72 % were female, and the mean age was 36.6 years (17 - 78 years). The average daily UAS and AAS value (mean ± standard deviation) were 2.1 ± 1.9 and 7.2 ± 3.3, respectively. Most CRUSE patients had poorly controlled disease, with mean UCT values of 7.0 ± 4.4 and mean AECT values of 8.1 ± 4.5. Conclusion: The first days of patients with CSU using CRUSE confirm the high need for an app that helps to monitor disease activity, impact, and control. The first results indicate low levels of disease control in most CRUSE users, with low UCT and AECT values. Future analyses will assess follow-up documentation data and evaluate the effects of treatment changes on CSU activity, impact, and control., (© 2023 Neisinger et al, HBKU Press.)

Background: Concern about disease exacerbations and fear of reactions after coronavirus disease 2019 (COVID-19) vaccinations are common in chronic urticaria (CU) patients and may lead to vaccine hesitancy., Objective: We assessed the frequency and risk factors of CU exacerbation and adverse reactions in CU patients after COVID-19 vaccination., Methods: COVAC-CU is an international multicenter study of Urticaria Centers of Reference and Excellence (UCAREs) that retrospectively evaluated the effects of COVID-19 vaccination in CU patients aged ≥18 years and vaccinated with ≥1 dose of any COVID-19 vaccine. We evaluated CU exacerbations and severe allergic reactions as well as other adverse events associated with COVID-19 vaccinations and their association with various CU parameters., Results: Across 2769 COVID-19-vaccinated CU patients, most (90%) received at least 2 COVID-19 vaccine doses, and most patients received CU treatment and had well-controlled disease. The rate of COVID-19 vaccination-induced CU exacerbation was 9%. Of 223 patients with CU exacerbation after the first dose, 53.4% experienced recurrence of CU exacerbation after the second dose. CU exacerbation most often started <48 hours after vaccination (59.2%), lasted for a few weeks or less (70%), and was treated mainly with antihistamines (70.3%). Factors that increased the risk for COVID-19 vaccination-induced CU exacerbation included female sex, disease duration shorter than 24 months, having chronic spontaneous versus inducible urticaria, receipt of adenovirus viral vector vaccine, having nonsteroidal anti-inflammatory drug/aspirin intolerance, and having concerns about getting vaccinated; receiving omalizumab treatment and Latino/Hispanic ethnicity lowered the risk. First-dose vaccine-related adverse effects, most commonly local reactions, fever, fatigue, and muscle pain, were reported by 43.5% of CU patients. Seven patients reported severe allergic reactions., Conclusions: COVID-19 vaccination leads to disease exacerbation in only a small number of CU patients and is generally well tolerated., (Copyright © 2023 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.)

Coronavirus disease 2019 (COVID-19) is a highly contagious viral disease caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). It has various effects on asthma, allergic rhinitis, atopic dermatitis, and urticaria and may change the course of the disease depending on the severity of the infection and control status of the disease. Conversely, these diseases may also impact the course of COVID-19. Patients with chronic urticaria and atopic dermatitis may have COVID-19-induced disease exacerbations and biological treatments reduce the risk of exacerbations. Poor asthma control is linked to severe COVID-19 while allergic asthma is associated with lower risk of death and a lower rate of hospitalization due to COVID-19 compared with nonallergic asthma. The use of intranasal corticosteroids is associated with lower rates of hospitalization due to COVID-19 in patients with allergic rhinitis, whereas the effect of inhaled corticosteroids is confounded by asthma severity. These observations reinforce the importance of keeping allergic diseases under control during pandemics. The use of biologicals during COVID-19 is generally regarded as safe, but more evidence is needed. The pandemic substantially changed the management of allergic disorders such as home implementation of various biologicals, allergen immunotherapy, food introduction, and increased use of telemedicine and even home management of anaphylaxis to reduce emergency department burden and reduce risk of infection. Physicians need to be aware of the potential impact of COVID-19 on allergic diseases and educate their patients on the importance of continuing prescribed medications and adhering to their treatment plans to maintain optimal control of their disease., (Copyright © 2023 American Academy of Allergy, Asthma & Immunology. All rights reserved.)

Background: Patients with chronic spontaneous urticaria (CSU) have spontaneous wheals (W), angioedema (AE), or both, for longer than 6 weeks. Clinical differences between patients with standalone W, standalone AE, and W and AE (W+AE) remain incompletely understood., Objective: To compare W, AE, and W+AE CSU patients regarding demographics, disease characteristics, comorbidities, disease burden, and treatment response., Methods: Baseline data from 3,698 CSU patients in the ongoing, prospective, international, multicenter, observational Chronic Urticaria REgistry (CURE) were analyzed (data cut: September 2022)., Results: Across all CSU patients, 59%, 36%, and 5% had W+AE, W, and AE, respectively. The W+AE patients, compared with W and AE patients, showed the lowest male-to-female ratio (0.33), higher rates of concomitant psychiatric disease (17% vs 11% vs 6%, respectively), autoimmune disease (13% vs 7% vs 9%, respectively), and nonsteroidal anti-inflammatory drug (NSAID) hypersensitivity (9% vs 5% vs 2%, respectively) and the highest disease impact. The W patients, compared with W+AE and AE patients, showed the lowest rates of concomitant hypertension (15% vs 21% vs 40%, respectively) and obesity (11% vs 16% vs 17%, respectively), the highest rate of concomitant inducible urticaria (24% vs 22% vs 6%, respectively), and shorter W duration. The AE patients, compared with W+AE and W patients, were older at disease onset, showed longer AE duration, and the best response to increased doses of H 1 -antihistamines (58% vs 24% vs 31%, respectively) and omalizumab (92% vs 67% vs 60%, respectively)., Conclusions: Our findings provide a better understanding of CSU phenotypes and may guide patient care and research efforts that aim to link them to pathogenic drivers., (Copyright © 2023. Published by Elsevier Inc.)

Purpose of Review: The purpose of this review is to provide an overview of the recent advancements and relevance of the autoimmune theories in chronic spontaneous urticaria (CSU)., Recent Findings: Two primary types of autoimmunity, Type I and Type IIb, have emerged as major contributors to CSU, characterized by immunoglobulin E (IgE) and immunoglobulin G (IgG) autoantibodies, respectively. Genetic evidence supports the notion that CSU shares more similarities with other autoimmune diseases rather than atopic diseases. Novel autoallergens such as FcεRI and tissue transglutaminase have been identified, contributed to our understanding of autoimmune mechanisms. Furthermore, the potential overlap between Type I and Type IIb autoimmunity has been recognized. Evaluating the autoimmune status of CSU patients through biomarkers and understanding their clinical implications is vital for effective management. For instance, CSU patients with Type IIb autoimmunity, with or without coexisting Type I autoimmunity, may exhibit resistance to H1-antihistamines and omalizumab treatment but could potentially respond well to cyclosporine or Bruton's tyrosine kinase inhibitors., Summary: Further investigations are needed to explore new autoallergens and autoantibodies in CSU, establishing their connection to the development of autoimmunity. The efficacy of novel drugs targeting different mechanisms should be examined to determine their responses in both autoimmune CSU and nonautoimmunity-related CSU., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Introduction: The integrated care pathways for atopic dermatitis (AD-ICPs) aim to bridge the gap between existing AD treatment evidence-based guidelines and expert opinion based on daily practice by offering a structured multidisciplinary plan for patient management of AD. ICPs have the potential to enhance guideline recommendations by combining interventions and aspects from different guidelines, integrating quality assurance, and describing co-ordination of care. Most importantly, patients can enter the ICPs at any level depending on AD severity, resources available in their country, and economic factors such as differences in insurance reimbursement systems., Methods: The GA 2 LEN ADCARE network and partners as well as all stakeholders, abbreviated as the AD-ICPs working group, were involved in the discussion and preparation of the AD ICPs during a series of subgroup workshops and meetings in years 2020 and 2021, after which the document was circulated within all GAL 2 EN ADCARE centres., Results: The AD-ICPs outline the diagnostic procedures, possible co-morbidities, different available treatment options including differential approaches for the pediatric population, and the role of the pharmacists and other stakeholders, as well as remaining unmet needs in the management of AD., Conclusion: The AD-ICPs provide a multidisciplinary plan for improved diagnosis, treatment, and patient feedback in AD management, as well as addressing critical unmet needs, including improved access to care, training specialists, implementation of educational programs, assessment on the impact of climate change, and fostering a personalised treatment approach. By focusing on these key areas, the initiative aims to pave the way for a brighter future in the management of AD., (© 2023 The Authors. Clinical and Translational Allergy published by John Wiley & Sons Ltd on behalf of European Academy of Allergy and Clinical Immunology.)

Background: Chronic spontaneous urticaria (CSU) and urticarial vasculitis (UV) share several clinical features including the occurrence of wheals. As of yet, the criteria for differentiating the 2 disorders are not clearly defined., Objective: Here, we aimed to identify differences, similarities, and the likelihood for specific clinical features in patients with UV versus those with CSU., Methods: Across 10 Urticaria Centers of Reference and Excellence, 106 patients with skin biopsy-confirmed UV and 126 patients with CSU were prospectively recruited to complete a questionnaire on the clinical features, course, and response to treatment of their disease., Results: As compared with CSU, patients with UV more often experienced postinflammatory skin hyperpigmentation, wheals of ≥24-hour duration, eye inflammation, and fever (6.9, 4.0, 3.6, and 2.4 times, respectively). Clinical features that increased the risk for UV diagnosis when present at the onset of disease included wheals of ≥24-hour duration (7.3-fold), pain of the skin (7.0-fold), postinflammatory hyperpigmentation (4.1-fold), and fatigue (3.1-fold). The diagnostic delay was markedly longer for normocomplementemic UV as compared with hypocomplementemic UV and CSU (21 vs 5 vs 6 months, respectively). Oral corticosteroids and omalizumab were the most effective treatments in patients with UV and CSU, respectively. Patients with UV showed a higher need for immunosuppressive and anti-inflammatory therapies than patients with CSU., Conclusions: Long wheal duration, skin pain and hyperpigmentation, and systemic symptoms point to UV rather than CSU as the underlying disease and should prompt further diagnostic workup including a skin biopsy., (Copyright © 2023 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.)

Since more than a century ago, there has been awareness of the connection between viral infections and the onset and exacerbation of urticaria. Our knowledge about the role of viral infection and vaccination in acute and chronic urticaria improved as a result of the COVID-19 pandemic but it has also highlighted knowledge gaps. Viral infections, especially respiratory tract infections like COVID-19, can trigger the onset of acute urticaria (AU) and the exacerbation of chronic urticaria (CU). Less frequently, vaccination against viruses including SARS-CoV-2 can also lead to new onset urticaria as well as worsening of CU in minority. Here, with a particular focus on COVID-19, we review what is known about the role of viral infections and vaccinations as triggers and causes of acute and chronic urticaria. We also discuss possible mechanistic pathways and outline the unmet needs in our knowledge. Although the underlying mechanisms are not clearly understood, it is believed that viral signals, medications, and stress can activate skin mast cells (MCs). Further studies are needed to fully understand the relevance of viral infections and vaccinations in acute and chronic urticaria and to better clarify causal pathways.

Background and Objective: Chronic spontaneous urticaria (CSU) is a distressing disease. We report real-world data from the global Chronic Urticaria Registry (CURE) about associations between various CSU states and sleep impairment, plus important health-related quality-of-life (HRQoL) outcomes and compared different methods to assess CSU states., Methods: CURE data were collected at baseline and 6-monthly follow-ups (FU). Assessments included CSU states using the Urticaria Control Test (UCT), weekly Urticaria Activity Score (UAS7), and Physician Global Assessment (PhyGA) of treatment response. Complete response to treatment (CR, UAS7 = 0), complete control of disease (CC, UCT = 16), and PhyGA = CR were assessed, plus the Dermatology Life Quality Index and the Chronic Urticaria Quality-of-Life Questionnaire (CU-Q 2 oL) sleep domain., Results: Overall, 2078 patients were included. At baseline, 9.8%, 17.9%, and 42.3% of patients had UCT = 16, UAS7 = 0, or PhyGA = CR, respectively, which increased at FU1 and FU2. Patients with higher UCT scores had better sleep and HRQoL. The presence of angioedema without wheals, episodic disease, omalizumab treatment, and male sex were associated with CC (P < .05). Among 469 patients who achieved CC or CR, 16.4% (n = 77) showed CC or CR with all 3 instruments. Agreement between UCT = 16 and UAS7 = 0 measurements was moderate (κ = 0.581), but poor between UCT = 16 and PhyGA = CR (κ = 0.208)., Conclusions: Few patients had CR/CC of their CSU at baseline entry. Disease control strongly related to good sleep and better HRQoL; therefore, it is important to aim for CR in CSU treatment. Patient-reported UCT and UAS7 assessments demonstrated a more accurate measurement of CSU state versus physician assessments., (Copyright © 2022. Published by Elsevier Inc.)

Background: Chronic Spontaneous Urticaria (CSU) is characterized by recurrent wheals and/or angioedema for longer than 6-weeks. Guidelines recommend Omalizumab (Oma) as first-line and Cyclosporine-A (Cs-A) as second-line treatment in antihistamine resistant CSU. This step-wise algorithm might be time-consuming and costly., Objective: To determine indicators of response to Oma or Cs-A in CSU patients., Methods: We retrospectively analyzed data from seven centers in Turkey; the inclusion criteria for patients were to receive both Oma and Cs-A treatment (not concurrently) at some point in time during their follow-up. Clinical and laboratory features were compared between groups., Results: Among 110 CSU patients; 47 (42.7%) were Oma-responders, 15 (13.6%) were Cs-A-responders, and 24 (21.8%) were both Oma and Cs-A responders and 24 (21.8%) were non-responders to either drug. High CRP levels were more frequent in Cs-A-responders (72.7% vs. 40.3%; p = 0.055). Oma-responders had higher baseline UCT (Urticaria Control Test) scores (6 vs. 4.5; p = 0.045). Responders to both drugs had less angioedema and higher baseline UCT scores compared to other groups (33.3% vs. 62.8%; p = 0.01 and 8 vs. 5; p = 0.017). Non-responders to both drugs had an increased frequency in the female gender and lower baseline UCT scores compared to other groups (87.5% vs. 61.6%; p = 0.017 and 5 vs. 7; p = 0.06)., Study Limitations: Retrospective nature, limited number of patients, no control group, the lack of the basophil activation (BAT) or BHRA (basophil histamine release assay) tests., Conclusions: Baseline disease activity assessment, which considers the presence of angioedema and disease activity scores, gender, and CRP levels might be helpful to predict treatment outcomes in CSU patients and to choose the right treatment for each patient. Categorizing patients into particular endotypes could provide treatment optimization and increase treatment success., (Copyright © 2022. Published by Elsevier España, S.L.U.)

Background: Cold urticaria (ColdU), that is, the occurrence of wheals or angioedema in response to cold exposure, is classified into typical and atypical forms. The diagnosis of typical ColdU relies on whealing in response to local cold stimulation testing (CST). It can also manifest with cold-induced anaphylaxis (ColdA). We aimed to determine risk factors for ColdA in typical ColdU., Methods: An international, cross-sectional study COLD-CE was carried out at 32 urticaria centers of reference and excellence (UCAREs). Detailed history was taken and CST with an ice cube and/or TempTest ® performed. ColdA was defined as an acute cold-induced involvement of the skin and/or visible mucosal tissue and at least one of: cardiovascular manifestations, difficulty breathing, or gastrointestinal symptoms., Results: Of 551 ColdU patients, 75% (n = 412) had a positive CST and ColdA occurred in 37% (n = 151) of the latter. Cold-induced generalized wheals, angioedema, acral swelling, oropharyngeal/laryngeal symptoms, and itch of earlobes were identified as signs/symptoms of severe disease. ColdA was most commonly provoked by complete cold water immersion and ColdA caused by cold air was more common in countries with a warmer climate. Ten percent (n = 40) of typical ColdU patients had a concomitant chronic spontaneous urticaria (CSU). They had a lower frequency of ColdA than those without CSU (4% vs. 39%, p = .003). We identified the following risk factors for cardiovascular manifestations: previous systemic reaction to a Hymenoptera sting, angioedema, oropharyngeal/laryngeal symptoms, and itchy earlobes., Conclusion: ColdA is common in typical ColdU. High-risk patients require education about their condition and how to use an adrenaline autoinjector., (© 2021 The Authors. Allergy published by European Academy of Allergy and Clinical Immunology and John Wiley & Sons Ltd.)

This update and revision of the international guideline for urticaria was developed following the methods recommended by Cochrane and the Grading of Recommendations Assessment, Development and Evaluation (GRADE) working group. It is a joint initiative of the Dermatology Section of the European Academy of Allergology and Clinical Immunology (EAACI), the Global Allergy and Asthma European Network (GA²LEN) and its Urticaria and Angioedema Centers of Reference and Excellence (UCAREs and ACAREs), the European Dermatology Forum (EDF; EuroGuiDerm), and the Asia Pacific Association of Allergy, Asthma and Clinical Immunology with the participation of 64 delegates of 50 national and international societies and from 31 countries. The consensus conference was held on 3 December 2020. This guideline was acknowledged and accepted by the European Union of Medical Specialists (UEMS). Urticaria is a frequent, mast cell-driven disease that presents with wheals, angioedema, or both. The lifetime prevalence for acute urticaria is approximately 20%. Chronic spontaneous or inducible urticaria is disabling, impairs quality of life, and affects performance at work and school. This updated version of the international guideline for urticaria covers the definition and classification of urticaria and outlines expert-guided and evidence-based diagnostic and therapeutic approaches for the different subtypes of urticaria., (© 2021 GA²LEN. Allergy published by European Academy of Allergy and Clinical Immunology and John Wiley & Sons Ltd.)

Background: Patients with chronic urticaria (CU) are increasingly using information and communication technologies (ICTs) to manage their health. What CU patients expect from ICTs and which ICTs they prefer remains unknown. We assessed why CU patients use ICTs, which ones they prefer, and what drives their expectations and choices., Methods: In this cross-sectional study, 1841 patients across 17 countries were recruited at UCAREs (Urticaria Centers of Reference and Excellence). Patients with CU who were >12 years old completed a 23-item questionnaire., Results: Most patients were interested in receiving disease information (87.3%), asking physicians about CU (84.1%), and communicating with other patients through ICTs (65.6%). For receiving disease information, patients preferred one-to-one and one-to-many ICTs, especially web browsers. One-to-one ICTs were also the ICTs of choice for asking physicians about urticaria and for communicating with other patients, and e-mail and WhatsApp were the preferred ICTs, respectively. Many-to-many ICTs such as Facebook, Instagram, LinkedIn, and Twitter were least preferred for all 3 purposes. Living in rural areas and higher education were linked to higher odds of being interested in receiving disease information, asking physicians, and communicating with patients through ICTs., Conclusions: Most patients and especially patients with higher education who live in rural areas are interested in using ICTs for their healthcare, but prefer different ICTs for different purposes, ie, web browsers for obtaining information, e-mail for asking physicians, and WhatsApp for communicating with other patients. Our findings may help to improve ICTs for CU., Competing Interests: I Cherrez-Ojeda has no conflicts of interest. E Vanegas has no conflicts of interest. A Cherrez has no conflicts of interest. M Felix has no conflicts of interest. K Weller is or recently was a speaker and/or advisor for, and/or has received research funding from: Biocryst, CSL Behring, Dr. Pfleger, FAES, Moxie, Novartis, Shire/Takeda, and Uriach. M Magerl is or recently was a speaker and/or advisor for, and/or has received research funding from Biocryst, CSL Behring, Kalvista Pharmaceuticals, Moxie, Novartis, Pharming, and Shire/Takeda. RR Maurer has no conflicts of interest. VL Mata has no conflicts of interest. A Kasperska-Zajac has no conflicts of interest. A Sikora has no conflicts of interest. D Fomina is or recently was a speaker and/or advisor for, and/or has received research funding from: AstraZeneca, CSL Behring, Glaxo SmithKline, MSD, Novartis, Sanofi, and Shire/Takeda. E Kovalkova has no conflicts of interest. K Godse has no conflicts of interest. N Dheeraj Rao has no conflicts of interest. M Khoshkhui has no conflicts of interest. S Rastgoo has no conflicts of interest. RFJ Criado has no conflicts of interest. M Abuzakouk has no conflicts of interest. D Grandon has no conflicts of interest. MBA van Doorn is or recently was a speaker and/or advisor for, and/or has received research funding from Abbvie, BMS, Celgene, Janssen Cilag, LEO Pharma, Lilly, MSD, Novartis, Pfizer, and Sanofi-Genzyme. S Valle has no conflicts of interest. E Magalhães de Souza Lima has no conflicts of interest. SF Thomsen is or recently was a speaker and/or advisor for, and/or has received research funding from: Abbvie, AstraZeneca, Celgene, Eli Lilly, Janssen, LEO Pharma, Novartis, Pierre Fabre, Roche, Sanofi, and UCB. GD Ramón has no conflicts of interest. EE Matos Benavides has no conflicts of interest. A Bauer is or recently was a speaker and/or advisor for, and/or has received research funding from Novartis, Sanofi, Genentech, Leo Pharma and Shire/Takeda. Ana M Giménez-Arnau has held roles as a Medical Advisor for Sanofi, Uriach, Genentech, Novartis, Amgen , ThemoFisher, Roche, Almirall, and has research grants supported by Instituto Carlos III- FEDER, Novartis, and Uriach; she also participates in educational activities for Almirall, Genentech, Glaxo SmithKline, LEO Pharma, Menarini, MSD, Novartis, Sanofi, Avene and Uriach. E Kocatürk is or recently was a speaker and/or advisor for Bayer, Novartis, and Sanofi. C Guillet has no conflicts of interest. JI Larco is or recently was a speaker and/or advisor for: FAES, Novartis, and Sanofi. Z-T Zhao has no conflicts of interest. M Makris is or recently was a speaker and/or advisor for, and/or has received research funding from AstraZeneca, Chiesi, Glaxo SmithKline, Novartis, and Sanofi. C Ritchie has no conflicts of interest. P Xepapadak reports personal fees from Galenica Greece, Glaxo SmithKline, Nestle, Novartis, Nutricia, and Uriach, outside the submitted work. LF Ensina is or recently was a speaker and/or advisor for, and/or has received research funding from Novartis, Sanofi, and Takeda. S Cherrez has no conflicts of interest. M Maurer is or recently was a speaker and/or advisor for, and/or has received research funding from: Allakos, Alnylam, Aralez, AstraZeneca, Biocryst, Blueprint, CSL Behring, FAES, Genentech, Kalvista Pharmaceuticals, LEO Pharma, Menarini, Moxie, MSD, Novartis, Pharming, Pharvaris, Roche, Sanofi, Shire/Takeda, UCB, and Uriach., (© 2021 The Author(s).)

Chronic urticaria and angioedema are diseases often managed by Allergy and Immunology specialists. Recent international guidelines have outlined a stepwise approach to management of patients using dose escalation of second-generation antihistamines followed by use of omalizumab and finally cyclosporine in more refractory cases. In select patients (those with refractory chronic urticaria), nonbiologic alternative medications with anti-inflammatory or immunosuppressant activity may be considered. Angioedema without wheals may have several different pathophysiologic mechanisms. Optimal management of mast cell-mediated angioedema is less clear but is often managed similar to chronic spontaneous urticaria. Drug-induced angioedema due to angiotensin-converting enzyme inhibitors is a common cause of angioedema in the emergency department. Although bradykinin is thought to be a primary mediator for this type of angioedema, studies of targeted therapies have been generally disappointing. In contrast, several targeted therapies have been proven successful using acute and preventive approaches for management of hereditary angioedema. Further developments, including novel biologics, novel oral therapies, and gene therapy approaches, may hopefully continue to broaden therapeutic options to ensure optimal individual management of patients with hereditary angioedema., (Copyright © 2021 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.)

Background: Chronic urticaria (CU) is characterized by itchy recurrent wheals, angioedema, or both for 6 weeks or longer. CU can greatly impact patients' physical and emotional quality of life. Patients with chronic conditions are increasingly seeking information from information and communications technologies (ICTs) to manage their health. The objective of this study was to assess the frequency of usage and preference of ICTs from the perspective of patients with CU., Methods: In this cross-sectional study, 1800 patients were recruited from primary healthcare centers, university hospitals or specialized clinics that form part of the UCARE (Urticaria Centers of Reference and Excellence) network throughout 16 countries. Patients were >12 years old and had physician-diagnosed chronic spontaneous urticaria (CSU) or chronic inducible urticaria (CIndU). Patients completed a 23-item questionnaire containing questions about ICT usage, including the type, frequency, preference, and quality, answers to which were recorded in a standardized database at each center. For analysis, ICTs were categorized into 3 groups as follows: one-to-one: SMS, WhatsApp, Skype, and email; one-to-many: YouTube, web browsers, and blogs or forums; many-to-many: Instagram, Twitter, Facebook, and LinkedIn., Results: Overall, 99.6% of CU patients had access to ICT platforms and 96.7% had internet access. Daily, 85.4% patients used one-to-one ICT platforms most often, followed by one-to-many ICTs (75.5%) and many-to-many ICTs (59.2%). The daily ICT usage was highest for web browsers (72.7%) and WhatsApp (70.0%). The general usage of ICT platforms increased in patients with higher levels of education. One-to-many was the preferred ICT category for obtaining general health information (78.3%) and for CU-related information (75.4%). A web browser (77.6%) was by far the most commonly used ICT to obtain general health information, followed by YouTube (25.8%) and Facebook (16.3%). Similarly, for CU-specific information, 3 out of 4 patients (74.6%) used a web browser, 20.9% used YouTube, and 13.6% used Facebook. One in 5 (21.6%) patients did not use any form of ICT for obtaining information on CU. The quality of the information obtained from one-to-many ICTs was rated much more often as very interesting and of good quality for general health information (53.5%) and CU-related information (51.5%) as compared to the other categories., Conclusions: Usage of ICTs for health and CU-specific information is extremely high in all countries analyzed, with web browsers being the preferred ICT platform., Competing Interests: M Maurer is or recently was a speaker and/or advisor for, and/or has received research funding from: Allakos, Alnylam, Aralez, AstraZeneca, Biocryst, Blueprint, CSL Behring, FAES, Genentech, Kalvista Pharmaceuticals, LEO Pharma, Menarini, Moxie, MSD, Novartis, Pharming, Pharvaris, Roche, Sanofi, Shire/Takeda, UCB, and Uriach. K Weller is or recently was a speaker and/or advisor for, and/or has received research funding from: Biocryst, CSL Behring, Dr. Pfleger, FAES, Moxie, Novartis, Shire/Takeda, and Uriach. M Magerl is or recently was a speaker and/or advisor for, and/or has received research funding from Biocryst, CSL Behring, Kalvista Pharmaceuticals, Moxie, Novartis, Pharming, and Shire/Takeda. RR Maurer has no conflicts of interest. E Vanegas has no conflicts of interest. M Felix has no conflicts of interest. A Cherrez has no conflicts of interest. VL Mata has no conflicts of interest. A Kasperska-Zajac has no conflicts of interest. A Sikora has no conflicts of interest. D Fomina is or recently was a speaker and/or advisor for, and/or has received research funding from: AstraZeneca, CSL Behring, Glaxo SmithKline, MSD, Novartis, Sanofi, and Shire/Takeda. E Kovalkova has no conflicts of interest. K Godse has no conflicts of interest. N Dheeraj Rao has no conflicts of interest. M Khoshkhui has no conflicts of interest. S Rastgoo has no conflicts of interest. RFJ Criado has no conflicts of interest. M Abuzakouk has no conflicts of interest. D Grandon has no conflicts of interest. M van Doorn is or recently was a speaker and/or advisor for, and/or has received research funding from Abbvie, BMS, Celgene, Janssen Cilag, LEO Pharma, Lilly, MSD, Novartis, Pfizer, and Sanofi-Genzyme. S Valle has no conflicts of interest. E Magalhães de Souza Lima has no conflicts of interest. SF Thomsen is or recently was a speaker and/or advisor for, and/or has received research funding from: Abbvie, AstraZeneca, Celgene, Eli Lilly, Janssen, LEO Pharma, Novartis, Pierre Fabre, Roche, Sanofi, and UCB. GD Ramón has no conflicts of interest. EE Matos Benavides has no conflicts of interest. A Bauer has no conflicts of interest. Ana M Giménez-Arnau has held roles as a Medical Advisor for Sanofi and Uriach, and has research grants supported by Instituto Carlos III- FEDER, Novartis, and Uriach; she also participates in educational activities for Almirall, Genentech, Glaxo SmithKline, LEO Pharma, Menarini, MSD, Novartis, Sanofi, and Uriach. E Kocatürk is or recently was a speaker and/or advisor for Bayer, Novartis, and Sanofi. C Guillet has no conflicts of interest. JI Larco is or recently was a speaker and/or advisor for: FAES, Novartis, and Sanofi. Z-T Zhao has no conflicts of interest. M Makris is or recently was a speaker and/or advisor for, and/or has received research funding from AstraZeneca, Chiesi, Glaxo SmithKline, Novartis, and Sanofi. C Ritchie has no conflicts of interest. P Xepapadak reports personal fees from Galenica Greece, Glaxo SmithKline, Nestle, Novartis, Nutricia, and Uriach, outside the submitted work. LF Ensina is or recently was a speaker and/or advisor for, and/or has received research funding from Novartis, Sanofi, and Takeda. S Cherrez has no conflicts of interest. I Cherrez-Ojeda has no conflicts of interest., (© 2020 Published by Elsevier Inc. on behalf of World Allergy Organization.)

Chronic spontaneous urticaria (CSU) is defined as the spontaneous development of itchy hives and/or angioedema due to known or unknown causes that last for at least 6 weeks. At any given time, CSU is believed to affect 0.5-1% of the global population. Omalizumab (a recombinant, humanized anti-immunoglobulin-E antibody) is the only approved treatment for antihistamine refractory CSU. However, ~ 30% of patients remain symptomatic at licensed doses of omalizumab 150 mg and 300 mg, even after a treatment period of over 6 months. In the recent years, there have been several studies on updosing of the drug, suggesting that the individualized approach for urticaria treatment with omalizumab is useful. In this article, we provide an overview of these studies and the real-world data on omalizumab updosing as it became necessary to obtain complete CSU symptom control in a proportion of patients. Published observational studies (from June 2003 to October 2019) on the updosing of omalizumab in CSU were identified using PubMed and Ovid databases. Reports mainly show that updosing/dose adjustment evaluated with the assessment of disease activity (Urticaria Activity Score) and control (Urticaria Control Test) achieves better clinical response to omalizumab with a good safety profile in a pool of patients with CSU. These real-world data will provide an overview of updosing of omalizumab in CSU and aid in setting informed clinical practice treatment expectations.

Background: Urticarial vasculitis (UV) is a rare type of leukocytoclastic vasculitis characterized by long lasting urticarial skin lesions and poor response to treatment. As of yet, no clinical guidelines, diagnostic criteria, or treatment algorithms exist, and the approaches to the diagnostic workup and treatment of UV patients may differ globally. We conducted an online survey to examine how UV patients are diagnosed and treated by international specialists and to reveal the greatest challenges in managing UV patients worldwide., Methods: Distribution of the questionnaire included an email to individuals in the World Allergy Organization (WAO) database, with no restrictions applied to the specialty, affiliation, or nationality of the participants (November 2018). The email contained a link (Internet address) to the online questionnaire. Responses were anonymous. The link to the questionnaire was further sent to the network of Urticaria Centers of Reference and Excellence (UCARE) in the Global Allergy and Asthma European Network (GA 2 LEN) as well as to the Turkish Dermatology Society and the Japanese Society of Allergology, who distributed the link to their members. In addition, the survey link was posted online in the group of the Russian Society of Allergologists and Immunologists., Results: We received 883 completed surveys from physicians in 92 countries. UV was reported to be rare in clinical practice, with an average of 5 patients per physician per year. More than two-thirds of physicians reported wheals, burning of the skin, and residual hyperpigmentation in 60-100% of UV patients. The most frequently reported reason for receiving referrals of patients with UV was to establish the diagnosis. The most important features for establishing the diagnosis of UV were wheals of longer than 24 hours duration (72%), the results of skin biopsy (63%), and post-inflammatory hyperpigmentation (46%). The most common tests ordered in UV patients were complete blood count, erythrocyte sedimentation rate, C-reactive protein, complement components, antinuclear antibodies, and skin biopsy. Physicians considered UV to be of unknown cause in most patients, and drugs and systemic lupus erythematosus to be the most common identifiable causes. Two of 3 physicians reported that they use second-generation antihistamines in standard dose as the first-line therapy in patients with UV. The greatest perceived challenges in the management of UV were the limited efficacy of drugs and the absence of clinical guidelines and treatment algorithms., Conclusions: UV is a challenging disease. Skin biopsy, a gold standard for UV diagnosis, is not performed by many physicians. This may lead to misdiagnosis of UV, for example, as chronic spontaneous urticaria, and to inadequate treatment. International consensus-based recommendations for the classification of UV and the diagnostic workup and treatment, as well as prospective studies evaluating potentially safe and effective drugs for the treatment of UV, are necessary., Competing Interests: The authors declare that they have no relevant conflicts of interest in relation to this work., (© 2020 The Authors.)

Background: The urticaria control test (UCT) is a questionnaire designed to determine if chronic urticaria (CU) is controlled or not and to aid therapeutic decision-making. It collects retrospective information about the symptoms and quality of life impairment over the last 4 weeks. The current study aimed to investigate the validity, reliability and sensitivity to change of the Turkish version of the UCT. We also evaluated its correlation with other tools and compared the UCT results of patients with chronic spontaneous urticaria (CSU) and patients with chronic inducible urticaria (CINDU)., Methods: Following forward/backward translation and cognitive debriefing, the Turkish version of the UCT was used in 81 CSU and 78 CINDU patients. Dermatology life quality index (DLQI), Chronic urticaria quality of life questionnaire (CU-Q2oL), urticaria activity score (UAS), patients' and physicians' global assessment visual analog scores and Likert scales were used at baseline and after four weeks to assess quality of life impairment, disease activity and disease control. Statistical analysis to determine the validity and reliability of the Turkish version of the UCT as well as comparison between CINDU and CSU patients were performed., Results: Duration of disease was longer, disease control was poorer and severe complaints were more frequent in CINDU patients (duration of disease: 36.3 (24) ± 49.1 vs 31.5 (9) ± 67.9, p = .007, UCT baseline: 8.4 (8) ± 3.4 vs 10.4 (11) ± 3.9, p = .001 and patient's global assessment Likert scale severe complaints: 6 vs 15, p < .001, respectively). The UCT showed excellent internal consistency for CSU and a minimally acceptable consistency for CINDU ( Cronbach's α 0.89 for CSU versus 0.68 for CINDU). It showed strong correlation with CU-Q2oL but a moderate correlation with DLQI (r = -0.649, P < .001 and r = -0.545, P < .001, respectively). It was able to discriminate between patients with different disease control and was sensitive to detect changes in the disease control in both groups. The minimally important difference of the UCT was found to be 3., Conclusions: The Turkish version of the UCT is a valid and reliable tool for the management of CU patients and can be used both in CSU and CINDU patients to determine if the treatment is sufficient and if disease activity and quality of life impairment are under control or not.

Background: The recommended treatment approach in chronic inducible urticarias (CIndU) is the same as that for chronic spontaneous urticaria (CSU). But there is a lack of controlled trials assessing efficacy of available treatment options., Objective: We aimed to evaluate the efficacy of treatment algorithm recommended by the guidelines and comparison of treatment responses in CIndU vs CSU., Methods: This prospective parallel group controlled study included 70 CIndU and 66 CSU patients. The same treatment algorithm recommended by the European Academy of Allergology and Clinical Immunology/Global Allergy and Asthma European Network/European Dermatology Forum/World Allergy Organization (EAACI/GA 2 LEN/EDF/WAO) was implemented to both CSU and CIndU patients. Treatment responses of the groups were evaluated with urticaria control test (UCT) and dermatology life quality questionnaire (DLQI) scores at the 0, 4, 8, 12 and 24th weeks for CIndU and 0, 4, 12 and 24 weeks for CSU., Results: Fourteen patients (20,9%) with CIndU and 25 (37,9%) with CSU responded to standard doses of H1-AHs which was significantly higher in the CSU group (p=0,031, p<0,05). Patients with CIndU and CSU showed statistically similar responses to 2nd line treatments (combining or updosing AHs) (p=0,979; p>0,05). Twenty-seven (40,3%) of CIndU patients and 21 (31,8%) of CSU patients were diagnosed as AH-resistant urticaria. Omalizumab was administered to 15 CSU patients and 17 CIndU patients. Response rates to omalizumab were similar in both groups. Total response rates increased from 37,9% (n:25) to 68,2% (n:45) with the 2nd line treatments in CSU group while it increased from 20,9% (n:14) to 59,7% (n:40) in CIndU group. When omalizumab was introduced to AH-refractory cases as a 3rd line treatment, total response rates evaluated at the 12th week were 76,1% (n:51) in patients with CIndU and 83,3% (n:55) in CSU. Continuing omalizumab treatment for 24 weeks increased response rates in patients who were unresponsive at week 12., Conclusion: CIndU seem to be more resistant to standard doses of AHs and higher doses of AHs are required for the control of symptoms. The same guidelines for CSU may be implemented to patients with CIndU., (Copyright © 2017 Japanese Society for Investigative Dermatology. Published by Elsevier B.V. All rights reserved.)