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A clear disease classification schema coupled with an understanding of the specific mechanisms involved in the different types of angioedema without hives informs the diagnostic assessment. The recommended approach involves several key steps. Foremost is the recognizing of the clinical clues which allow for the differentiation of mast cell-mediated disorders from bradykinin-mediated angioedema. Enhanced vascular permeability related to bradykinin is of critical importance to identify given the implications for disease morbidity and risk of mortality. The ability to efficiently categorize and diagnose all forms of angioedema results in improved patient outcomes., Competing Interests: Disclosure The authors have nothing to disclose., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Chronic inducible urticaria (CIndU) is characterized by the appearance of hives (urticaria) and/or angioedema in response to specific triggers or stimuli. For accurate diagnosis, anamnesis-driven specific, and if available, standardized trigger testings, as well as patient reported outcomes, should be applied. The currently recommended treatment algorithm is the same as for chronic spontaneous urticaria but is largely off-label for CIndU. New, and possibly more disease-specific, treatment options are needed for CIndU patients, who are often severely impacted by their disease. Several clinical trials are currently ongoing., Competing Interests: Disclosure S. Altrichter is or recently was a speaker and/or advisor for and/or has received research funding from AstraZeneca, United Kingdom, Allakos, BioCryst, United States, Blueprint, GSK, LEO Pharma, Lilly, LEO Pharma, Denmark, Moxie, Novartis, Switzerland, Phavaris, Takeda, Thermo Fisher, United States and Sanofi, France. D. Ritzel has no potential conflicts of interest to declare., (Copyright © 2024 Elsevier Inc. All rights reserved.)

The role of contact system activation has been clearly established in the pathogenesis of hereditary angioedema due to C1 inhibitor deficiency (HAE-C1INH). C1 inhibitor (C1INH)-protease complexes, levels of functional C1INH, plasma kallikrein activation, and cleavage of high-molecular-weight kininogen have each been associated with disease activity. More recently, HAE with normal levels of C1INH (HAE-nl-C1INH) has been recognized. Six genetic mutations have been identified which are linked to HAE-nl-C1INH phenotypes. The majority of individuals with HAE-nl-C1INH fall into the unknown category. There is substantial evidence that bradykinin generation underlies the recurrent attacks of swelling in some of these cohorts., Competing Interests: Disclosure None., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Competing Interests: Disclosure J. Stitt reports receiving grant support from Genentech.

Background: Angioedema (AE) is defined as localized, self-limited swelling of subcutaneous tissues and mucosa. Objective: The aim of this study was to compare the phenotypic characteristics of patients with AE without wheals. Methods: This prospective study included adult patients with recurrent AE without wheals. Demographic and laboratory data of the patients were recorded in the patient file when they presented to the outpatient clinic between August 2018 and August 2020. The patients were contacted by phone to evaluate whether their AE had gone into remission between October 2023 and January 2024. The phenotypic characteristics of AE subtypes were compared. Results: The study included a total of 143 patients. The average age, age of onset of AE, rates of diabetes mellitus, hypertension and coronary artery disease were higher in the patients with angiotensin-converting enzyme inhibitor (ACEI) use related acquired AE (AAE) (AAE-ACEI). The rates of allergic rhinitis, drug allergy, atopy, and aeroallergen sensitivity, and the median total immunoglobulin E level were higher in patients with idiopathic histaminergic AAE (AAE-IH). The rate of face and/or perioral AE attacks was higher in the patients with AAE-ACEI, AAE-IH, and idiopathic non-histaminergic AAE. The rate of AE attacks in limbs, abdominal, genital and other parts of the body was higher in patients with hereditary AE (HAE). The baseline AE activity score was lower in the patients with AAE-IH and higher in the patients with HAE. In long-term follow-up, the remission rate of AE attacks was significant higher in patients with AAE-ACEI and AAE-IH. Conclusion: The phenotypic characteristic features of Turkish patients with AE without wheals may vary, depending on the underlying AE pathogenesis. C1 inhibitor level and function, complement C4 and C1q, and genetic tests contributed to the diagnosis; other laboratory tests did not contribute to the diagnosis.

Urticaria and angioedema are caused by immunoglobulin E- and non-immunoglobulin E-mediated release of histamine and other inflammatory mediators from mast cells and basophils. Diagnosis is made clinically, and anaphylaxis must be ruled out if urticaria or angioedema is present. A limited nonspecific laboratory workup should be considered unless elements of the history or physical examination suggest specific underlying conditions. The mainstay of treatment is avoidance of triggers when and if triggers are identified. The first-line pharmacotherapy is second-generation H1 antihistamines, which can be titrated to greater than standard doses., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Sea anemones are marine animals that can produce toxins causing severe angioedema. Swimmers and divers should be aware of sea anemone species that can cause local and systemic toxic reactions and avoid indirect or direct skin contact. High index of suspicion, full laboratory workup, and treatment with steroids and antibiotics are imperative for an uneventful recovery., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Initiation of the bradykinin generation cascade is responsible for the occurrence of attacks in some types of angioedema without wheals. Hereditary angioedema due to C1 inhibitor deficiency (HAE-C1-INH) is one such clinical entity. In this paper, we explore the existing evidence that mast cells (MCs) degranulation may contribute to the activation of the kallikrein-kinin system cascade, followed by bradykinin formation and angioedema. We present the multidirectional effects of MC-derived heparin and other polyanions on the major components of the kinin-kallikrein system, particularly on the factor XII activation. Although, bradykinin- and histamine-mediated symptoms are distinct clinical phenomena, they share some common features, such as some similar triggers and a predilection to occur at sites where mast cells reside, namely the skin and mucous membranes. In addition, recent observations indicate a high incidence of hypersensitivity reactions associated with MC degranulation in the HAE-C1-INH patient population. However, not all of these can be explained by IgE-dependent mechanisms. Mast cell-related G protein-coupled receptor-X2 (MRGPRX2), which has recently attracted scientific interest, may be involved in the activation of MCs through a different pathway. Therefore, we reviewed MRGPRX2 ligands that HAE-C1-INH patients may be exposed to in their daily lives and that may affect MCs degranulation. We also discussed the known inter- and intra-individual variability in the course of HAE-C1-INH in relation to factors responsible for possible variability in the strength of the response to MRGPRX2 receptor stimulation. The above issues raise several questions for future research. It is not known to what extent a prophylactic or therapeutic intervention targeting the pathways of one mechanism (mast cell degranulation) may affect the other (bradykinin production), or whether the number of mast cells at a specific body site and their reactivity to triggers such as pressure, allergens or MRGPRX2 agonists may influence the occurrence of HAE-C1-INH attacks at that site., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The handling editor MR declared a past co-authorship with the author GP., (Copyright © 2024 Porebski, Dziadowiec, Rybka, Kitel and Kwitniewski.)

The aim of this prospective, randomized controlled clinical trial was to evaluate the effects of two methods of cold application on eye ecchymosis, periorbital edema, pain around the eyes and face, and patient comfort in postoperative rhinoplasty patients. Patients were randomly divided and evaluated in two groups: an ice in disposable latex gloves (IDLG) group and a cooling gel eye mask (CGEM) group. We used the CONSORT checklist to report the study. There were no significant differences between the groups in terms of age, gender, preoperative blood pressure, respiration, fever status, oxygen saturation, or postoperative vital signs. Patients in the IDLG group had significantly higher scores for pain around the eyes, facial pain, and periorbital edema on the first postoperative day, and significantly higher facial edema scores during the first postoperative hour (p ≤ .05). Patients in the CGEM group reported that they slept more comfortably (p ≤ .05). The results of our study showed that CGEMs reduce pain, periorbital edema, and facial edema after rhinoplasty., Competing Interests: The authors report no conflicts of interest., (Copyright © 2024 International Society of Plastic and Aesthetic Nurses. All rights reserved.)

Angioedema with eosinophilia (AE) is a rare disease of unknown etiology characterized by episodic (EAE) or nonepisodic AE (NEAE). Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) mRNA-based vaccines function as immunogens and intrinsic adjuvants and have been shown to be safe in large-scale trials. However, the long-term adverse reactions, especially those related to eosinophilic complications, have not been fully clarified. We herein report a case of self-limited but severe NEAE that developed in a young woman one week after receiving the second BNT162b2 mRNA vaccine. The symptoms that impaired her activities of daily living, such as edema, gradually resolved with supportive care over 10 weeks without corticosteroid treatment.

Most cases of angioedema are mast cell mediated. We present three patients with angioedema, who were admitted to our emergency room or outpatient clinic. One of them did have mast cell mediated angioedema, despite insufficient response to initial antihistamine treatment. The other patients had more rare cases of angioedema, i.e. hereditary angioedema with C1-inhibitor deficiency and angiotensin converting enzyme inhibitor associated angioedema. We discuss similarities and differences in symptoms, diagnosis and treatment between these causes of angioedema. We recommend keeping the differential diagnosis of angioedema in mind when a patient with angioedema is presented, including rarer pathophysiological explanations.

Background: Angioedema is the most common acute allergic presentation to emergency centres (EC), with hospitalisation rates increasing in high-income countries. Angioedema can complicate with life-threatening laryngeal obstruction. There are no local data; therefore, we aimed to characterise acute angioedema cases presenting to ECs and develop a simple management algorithm., Objective: To characterise the clinical presentation, management and outcomes of acute angioedema cases presenting to ECs. Based on these findings, we developed a management algorithm for acute angioedema to improve the care of acute angioedema in South Africa (SA)., Methods: We conducted a retrospective folder review of all patients admitted to Groote Schuur Hospital (tertiary) and Mitchells Plain District Hospital (secondary) ECs from 1  June 2018 to 31  June 2020. Using ICD-10 coding, folders of adults ≥18 years with possible angioedema presenting to the ECs were screened. An allergist extracted demographics, medical history, management and outcome data for each angioedema event., Results: A total of 142 acute angioedema episodes were included, with a median (interquartile range) age of 42 (28 - 58) years, and 62% of patients were female. The majority (124/142, 87%) of acute angioedema EC presentations involved swelling above the shoulders, with airway involvement in 20 (14%) patients, with two patients requiring intubation. Nineteen (13%) patients required admission, with five (26%) admitted to high care/intensive care. Drug-induced angioedema was the most common cause, with 64/142 (45%) linked to a known offending drug, 42/64 (65.6%) being angiotensin-converting enzyme inhibitor (ACE-I). Critical information to guide angioedema management, including past personal/family allergy history, and duration of angioedema prior to EC visit, was not recorded in 64.7% and 37.8% of EC records, respectively. Unnecessary treatment with corticosteroids or antihistamines occurred in 19/53 (36%) and 16/53 (30%) cases with bradykinin-mediated angioedema ACE-I angioedema and hereditary angioedema). Overall, only 36/142 (25%) of angioedema patients were connected to allergy care., Conclusion: Angioedema is the most common allergy presentation to two ECs in Cape Town, SA. Bradykinin-mediated angioedema secondary to ACE-I therapy is the single most common offender, and was not appropriately managed in more than a third of cases. Based on these findings, we have developed a management algorithm that easily stratifies patients into bradykinin or mast cell-mediated angioedema with a step-by-step management approach that is applicable to the SA context. Ongoing awareness and education on allergy emergencies are required to ensure accurate diagnosis of less common causes of angioedema (particularly bradykinin-mediated angioedema) and linkage to allergy specialist care.

Angioedema is a well-recognized and potentially lethal complication of angiotensin-converting enzyme inhibitor (ACEi) therapy. In ACEi-induced angioedema, bradykinin accumulates due to a decrease in its metabolism by ACE, the enzyme that is primarily responsible for this function. The action of bradykinin at bradykinin type 2 receptors leads to increased vascular permeability and the accumulation of fluid in the subcutaneous and submucosal space. Patients with ACEi-induced angioedema are at risk for airway compromise because of the tendency for the face, lips, tongue, and airway structures to be affected. The emergency physician should focus on airway evaluation and management when treating patients with ACEi-induced angioedema., Competing Interests: Disclosure The authors have nothing to disclose., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Scombroid poisoning, systemic mastocytosis, and hereditary alpha tryptasemia all present with episodes that resemble allergic reactions. Knowledge regarding systemic mastocytosis and hereditary alpha tryptasemia is quickly evolving. Epidemiology, pathophysiology, and strategies to identify and diagnose are discussed. Evidence-based management in the emergency setting and beyond is also explored and summarized. Key differences are described between these events and allergic reactions., Competing Interests: Disclosure The authors declare that they have no relevant or material financial interests that relate to the research described in this article., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Neurologic manifestations have been occasionally described in patients with bradykinin-mediated angioedema. The existing literature is currently limited to case series and case reports mainly described in the hereditary forms (HAE) concerning central nervous system (CNS) involvement. On the contrary, very little is known about peripheral and autonomic nervous system manifestations. CNS involvement in HAE may present with symptoms including severe headaches, visual disturbance, seizures, and various focal and generalized deficits. In addition, a stroke-like clinical picture may present in HAE patients. In turn, some drugs used in patients with cardiovascular and neurologic disorders, such as recombinant tissue plasminogen activator (r-tPA) and angiotensin-converting enzyme inhibitors (ACEI), may produce medication-induced angioedema, resulting in a diagnostic challenge. Finally, most patients with HAE have higher levels of psychological distress, anxiety, and depression. With this review, we aimed to provide an organized and detailed analysis of the existing literature on neurologic and psychiatric manifestations of HAE to shed light on these potentially invalidating symptoms and lay the foundation for further personalized diagnostic pathways for patients affected by this protean disease.

Background: Episodic angioedema with eosinophilia (EAE) (Gleich syndrome) is a rare disorder consisting of recurrent episodes of angioedema, hypereosinophilia, and frequent elevated serum IgM level., Methods: We conducted a retrospective multicenter nationwide study regarding the clinical spectrum and therapeutic management of patients with EAE in France., Results: A total of 30 patients with a median age at diagnosis of 41 years (range, 5-84) were included. The median duration of each crisis was 5.5 days (range, 1-90), with swelling affecting mainly the face and the upper limbs. Total serum IgM levels were increased in 20 patients (67%). Abnormal T-cell immunophenotypes were detected in 12 patients (40%), of whom 5 (17%) showed evidence of clonal T-cell receptor gamma locus gene (TRG) rearrangement. The median duration of follow-up was 53 months (range, 31-99). The presence of an abnormal T-cell population was the sole factor associated with a shorter time to flare (hazard ratio, 4.15; 95% confidence interval, 1.18-14.66; P = .02). At last follow-up, 3 patients (10%) were able to have all treatments withdrawn and 11 (37%) were in clinical and biologic remission with less than 10 mg of prednisone daily., Conclusion: EAE is a heterogeneous condition that encompasses several disease forms. Although patients usually respond well to glucocorticoids, those with evidence of abnormal T-cell phenotype have a shorter time to flare., (Copyright © 2019. Published by Elsevier Inc.)

Hereditary angioedema (HAE) is a rare autosomal dominant genetic disorder which causes bradykinin mediated angioedema. Although it can be life threatening, HAE may be underdiagnosed due to a lack of awareness of the disease and limited access to laboratory testing. Here, we report a case of HAE which was diagnosed only after the patient was referred for COVID-19 vaccination even though he had been experiencing recurrent angioedema for the past 30 years.

Background and Objectives . Acquired angioedema is a relatively common revelation accompanying some diseases such as autoimmune or cancer. The study aimed to assess the incidence of one subtype of angioedema-C1-INH-AAE (acquired angioedema with C1 inhibitor deficiency). Material and methods . The study was retrospective and based on 1 312 patients with a final diagnosis of breast cancer, colorectal cancer, or lung cancer: 723 women and 589 men with a mean age of 58.2 ± 13.5 years. The cancer diagnosis according to the ICD (International Classification of Diseases)-10 code, medical history including TNM (Tumour, Node, Metastasis) staging, histopathology, and assessment of the occurrence of C1-INH-AAE angioedema were analysed. Results . C1-INH-AAE occurred more often in patients with cancer than in the control group, as follows: 327 (29%) vs. 53 (6%) for p < 0.05. C1-INH-AAEs were observed most often in the group of patients diagnosed with breast cancer compared to colorectal and lung groups: 197 (37%) vs. 108 (26%) vs. 22 (16%) ( p < 0.05). A higher incidence of C1-INH-AAE was observed in the early stages of breast cancer. However, there was no relationship between the occurrence of C1-INH-AAE and the BRCA1 (Breast Cancer gene 1)/BRCA2 (Breast Cancer gene 2) mutation or histopathological types of breast cancer. Conclusion . Angioedema type C1-INH-AAE occurs more often in patients with selected neoplastic diseases, especially in the early stages of breast cancer.

This case reports a woman in her 40s with a history of allergic reaction to shellfish and iodine who presented with tongue angioedema, difficulty breathing and chest tightness after receiving the first dose of the Pfizer-BioNTech (BNT162b2) COVID-19 vaccine. Her angioedema remained for 10 days post-exposure to the vaccine, requiring 3 days of epinephrine infusion. She was discharged with advice to avoid further mRNA vaccines. This case highlights the increasing awareness needed of polyethylene glycol (PEG) allergy and the protracted nature of her reaction. A firm conclusion cannot be reached based on a single case report. More research is needed to understand whether there is a causal relationship between the BNT162b2 vaccine and PEG allergy. Awareness regarding PEG allergy and the complexities associated with it is important and needs to be raised due to its prevalent use in diverse industries., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Angioedema is an acute disorder that affects mucous membranes and the deepest layers of the skin along with underlying tissue, marked by rapid swelling, large welts, and pain. There are 3 major subtypes of angioedema: mast-cell mediated, bradykinin-mediated, and multifactorial or unclear mechanism subtype. The most common subtype of bradykinin-mediated angioedema is ACE-inhibitor induced, which disproportionately affects African-Americans. It is most often self-limiting and usually responds to the withdrawal of the offending agent. The prolonged duration of angioedema is uncommon in the absence of a persistent stimulus, though it is more likely when there is an abnormality of the metabolic pathways, such as in hereditary angioedema or other gene polymorphisms affecting the complement system. We present a case of severe angioedema that persisted for over a month and required a tracheostomy to manage the airway.

Angioedema without Urticaria: Medical History and Findings Abstract. Abstracts: We present the case of a woman with repeated attacks of angioedema without wheals. Given that there was no benefit from systemic steroid and antihistamines therapy, we interpretated the clinical picture as bradykinin- (and not histamine-) induced. Owing to the late onset and a negative family history, we suspected a case of angioedema due to acquired C1-INH deficiency. This hypothesis was later confirmed by specific hematological tests. We therefore started a specific prophylaxis and therapy in case of acute attacks.

Background: Acute urticaria and angioedema are emergency dermatological conditions associated with various etiologic factors., Objective: To determine the etiological causes in patients with acute urticaria and angioedema, and to investigate whether more than one etiological cause was present, along with the patients' laboratory values., Methods: The study was conducted in a tertiary hospital with one center. Etiological causes and laboratory parameters in 284 patients diagnosed with acute urticaria and angioedema were retrospectively studied., Results: A total of 284 patients were included in the study. The mean age of the patients was 42.7 ± 15.6 years, where 163 (57.4%) were women and 121 (42.6%) were men. Acute urticaria and angioedema occurred together in 149 (52.5%) patients. At least one precipitating factor among the predisposing risk factors was present in 220 (77.5%) patients, and more than one precipitating factor was found in 51 (18%) patients. Medication use was found in 157 (55.3%) patients and infection in 54 (19%). The development of urticaria after food consumption was noted in nine (3.2%) individuals. A history of infection and medication intake was present in 50 (17.6%) patients. A joint history of food consumption and medication intake was present in only one patient. Elevated C-reactive protein level was found in 178 (62.7%) patients and elevated anti-streptolysin O titer in 41 (14.4%) patients. Vitamin B12 deficiency was found in 116 (40.8%) patients and vitamin D deficiency in 254 (89.4%)., Conclusion: Acute urticaria and angioedema may occur as a result of multiple etiological factors, in which different triggers may be present simultaneously.

The prevalence of urticaria has been reported mostly in Europe and North America. However, precise information regarding its subtypes and clinical characteristics in primary care practice, especially in Asian countries, are scant. Patients with urticaria and/or angioedema who visited nine primary clinics of accredited dermatologists and allergologists in Japan were recruited from October to November 2020. The information of age, sex, disease duration, urticaria control test (UCT), and concomitant urticaria subtypes were collected. A total of 1061 patients participated. The number of patients was high in the 20 to 50 age groups with a peak in the 40s. The most frequent urticaria subtype was chronic spontaneous urticaria (CSU) followed by dermographism, acute spontaneous urticaria (ASU), angioedema, and cholinergic urticaria (CholU) (66.8%, 22.7%, 18.9%, 14.1% and 5.7% in all patients with urticaria). CSU development increased with age from the 20s to 50s, especially in females. Dermographism had a peak in the 40s. ASU had bimodal peaks in childhood and in the 30s. CholU was common in males in the 10-20s. Most angioedema patients were female with an increase in their 30s. Angioedema was solely present in 14 of 1061 participants (1.3%), while 136 (12.8%) had angioedema concomitant with urticaria. UCT showed poorly controlled urticaria with lower scores in patients with concomitant CSU and other subtypes than in those with CSU alone. Urticaria tends to develop in young to middle-aged females. The most common urticaria subtype is CSU, while the number of patients with CholU is high and that of angioedema is low in Japan., (© 2022 Japanese Dermatological Association.)

Abstract: Episodic hypereosinophilia and angioedema syndrome, also known as Gleich syndrome, is a rare entity characterized by recurrent episodes of eosinophilia, angioedema, urticaria, fever and weight gain with spontaneous resolution. It is classified as an idiopathic hypereosinophilic syndrome. Unlike other hypereosinophilic syndromes, it has a low risk for internal organ damage. We report the case of a 42-year-old male with a 28-year history of recurrent erythematous wheals and plaques and persistent hypereosinophilia. Physical examination revealed a well-defined subcutaneous nodule on his right lower limb that increased in size with each episode of angioedema. Histopathology evidenced a lipoma with intense eosinophil infiltration within the mature adipose tissue, while the specimen of the wheal revealed scarce perivascular and interstitial eosinophilic inflammatory infiltrate. Diagnosis of episodic angioedema with eosinophilia syndrome was made based on clinical and laboratory findings., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

C1-inhibitor deficiency is a rare disease which incorporates acute self-limiting intermittent swelling of the subcutaneous tissue and mucous membranes. Attacks most frequently affect the face and/or the upper airway. Isolated angioedema of the small bowel is an uncommon manifestation and often accompanied by diagnostic delay. In the present case, abdominal pain turned out to be the first and only utterance of an acquired C1-inhibitor deficiency, secondary to a splenic marginal zone lymphoma. Imaging showed wall thickening of the small intestine, ascites and splenomegaly. The abdominal pain and intestinal wall thickening with surrounding ascites on imaging spontaneously resolved each episode within 2-3 days. Gastrointestinal manifestations of angioedema may mimic an acute abdomen, and subsequently one-third of these patients undergo unnecessary surgery prior to a definite diagnosis. This emphasises the importance of considering the diagnosis in case of an 'extraordinary colic'., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

A previously healthy man in his 60s being worked up for splenomegaly presented to the emergency department with recurrent episodes of angioedema. Each episode was attributed to a precipitating cause, and consequently, the predisposing C1 esterase inhibitor (C1-INH) deficiency remained undiagnosed until the third presentation. The aetiology of acquired C1-INH deficiency would be primarily obscure and require further investigations to identify. A clonal B cell population was finally isolated by flow cytometry after multiple repeat marrow samples, and a diagnosis of splenic marginal zone lymphoma was subsequently reached. Response to single-agent rituximab was observed with resolution of splenomegaly, disappearance of the antibody and restoration of C1-INH levels., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Angioedema (AE) is a reason for emergency care when it is severe. Care is difficult when the diagnostic is not known before the attack: mast cell (MC) or bradykinin (BK) mediated. One is very common but often benign, the other rare but potentially fatal. The French national reference center of angioedema (CREAK) provides emergency physicians with a hotline and a guideline to help them manage their patients. This study aimed to describe the clinical features of AE episodes prompting a call on the CREAK hotline and classify patients depending on the suspected cause of the AE. This is a retrospective study between March and August 2019. Each physician calling on the CREAK hotline was asked to fill a clinical description form for the AE emergency. Known patients of CREAK was excluded. Eighty four patients were included. Forty one (48.8%) in the angiotensin converting enzyme inhibitors induced acquired angioedema (ACEi-AAE), 39 (46.4%) in the mast cell induced angioedema, and 4 (4.8%) in the Bradykinin mediated angioedema. The mast cell induced angioedema patients have more history of hives (29.3%) than ACEi-AAE (2.4%, P = .0004). ACEi-AAE mainly affected the tongue (58.5% vs 25.6%, P = .003) and larynx (29.3% vs 13%, P = .001). In 65.5% of cases, the etiological diagnosis was not mentioned by the appellant, but made by the hotline. In 31% of cases, the hotline suggested the administration of a specific treatment not previously provided by the caller. All the doctors who called the hotline appreciate this tele-expertise especially in case of ACEi-AAE presumptions. In addition to providing rapid AE expertise, this service also allows to educate physicians in the management of AE irrespective of its origin., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.)

Introduction: Ingestion of Anisakis is a common cause of allergic reactions to seafood in countries in which undercooked/raw seafood is part of gastronomic traditions. Despite current trends for the ingestion of raw/marinated/undercooked fish, the prevalence rate of anisakiasis and allergy to Anisakis is still considered to be low in Portugal. We aimed to review the current pathogenic mechanisms, the clinical and diagnostic approach of Anisakis allergy, and Anisakis-related eviction measures, while raising awareness to this problem., Material and Methods: Literature search in the MEDLINE and Scopus databases, regarding Anisakis allergy., Conclusion: Assessment of sensitization to Anisakis should be included in the workup study of urticaria/angioedema and anaphylaxis, as there is a rise in consumption of raw and undercooked fish. Ingestion of previously frozen and properly cooked fish appears to be safe for most patients who are allergic to Anisakis.

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.)