The Chronic Urticaria Registry: rationale, methods and initial implementation.

BACKGROUND: Chronic urticaria (CU) is a common disease, characterized by the recurrent appearance of wheals, angioedema or both for more than 6 weeks. Its underlying biology is not well understood, and many patients do not obtain sufficient relief from recommended treatments. Patient registries are rapidly growing as a form of research, because they can provide powerful, data-driven insights about the epidemiology of diseases, real-world effectiveness of treatments, rare patient types, safety monitoring, healthcare costs and opportunities for quality improvement of healthcare delivery. OBJECTIVES: The Chronic Urticaria Registry (CURE) has been designed to improve the scientific understanding, clinical treatment and healthcare planning of CU patients. This report describes the rationale, methods and initial implementation of this registry. METHODS: Chronic Urticaria Registry is an ongoing, prospective, international, multicentre, observational, voluntary registry of patients with CU. Participation in CURE is open to any physician treating CU patients, regardless of location, medical specialty or type of practice setting. CURE aims to collect data on all CU patients, with no intentional selection or exclusion criteria. It collects baseline and follow-up data on the patient's demographics, history, symptoms, trigger and risk factors, therapies and healthcare utilization. RESULTS: Chronic Urticaria Registry is a landmark achievement of the global urticaria medical community. As of 26 February 2020, 39 centres around the world have joined the registry and 35 have entered baseline data on a total of 2946 patients. Publications of this data will be forthcoming soon. CONCLUSIONS: Chronic Urticaria Registry is eagerly seeking the participation of more physicians and the support of more governmental, charitable and commercial sponsors from around the world. Here, in this paper, we invite other physicians to join this unique project to improve the lives of patients with CU.

[Omalizumab for the treatment of chronic urticaria: Real-life findings]. / Traitement de l'urticaire chronique par omalizumab : données en vie réelle.

OBJECTIVE: Assessment of the efficacy and safety of omalizumab in chronic urticaria refractory to conventional treatment (H1-antihistamines at high dosage and montelukast) in real-life practice. PATIENTS AND METHODS: A retrospective, descriptive, single-centre study was performed of the data for all patients presenting refractory chronic spontaneous urticaria or inducible urticaria and receiving omalizumab (300mg every four weeks) from November 2012 to June 2016. RESULTS: In all, 23 patients were included. Omalizumab led to complete or significant remission in 19 patients (83%) with chronic urticaria, with remission in 9 patients (47%) occurring within 72hours of the first injection. One patient had a partial response and 3 (13%) showed no response. Only 2 patients (9%) in complete remission stopped their treatment at 1 and 3 years. 52% of patients presented non-serious adverse events, which in one case resulted in treatment withdrawal. CONCLUSION: Omalizumab exhibited good real-life efficacy in a small series of chronic urticaria patients in France.

The EAACI/GA²LEN/EDF/WAO guideline for the definition, classification, diagnosis and management of urticaria.

This evidence- and consensus-based guideline was developed following the methods recommended by Cochrane and the Grading of Recommendations Assessment, Development and Evaluation (GRADE) working group. The conference was held on 1 December 2016. It is a joint initiative of the Dermatology Section of the European Academy of Allergology and Clinical Immunology (EAACI), the EU-founded network of excellence, the Global Allergy and Asthma European Network (GA²LEN), the European Dermatology Forum (EDF) and the World Allergy Organization (WAO) with the participation of 48 delegates of 42 national and international societies. This guideline was acknowledged and accepted by the European Union of Medical Specialists (UEMS). Urticaria is a frequent, mast cell-driven disease, presenting with wheals, angioedema, or both. The lifetime prevalence for acute urticaria is approximately 20%. Chronic spontaneous urticaria and other chronic forms of urticaria are disabling, impair quality of life and affect performance at work and school. This guideline covers the definition and classification of urticaria, taking into account the recent progress in identifying its causes, eliciting factors and pathomechanisms. In addition, it outlines evidence-based diagnostic and therapeutic approaches for the different subtypes of urticaria.

Chronic urticaria and hormones: Is there a link?

BACKGROUND: While the role of oestrogens in bradykinin angioedema (AE) has been clearly demonstrated, scarce data are available about the role of sex hormones in chronic urticaria (CU). OBJECTIVES: To gather information from a population of women with various forms of CU [chronic spontaneous urticaria (CSU), including a subtype of isolated histaminic AE and a classic subtype of association of wheals and AE, and exclusive inducible urticaria (IU)] about the impact of sex hormones and reproductive factors on their symptoms. METHODS: This was a cross-sectional study comprising interviews of 200 women consulting for CU at nine centres throughout France between May and July 2013. The dermatologists filled in an online questionnaire on the impact of reproductive factors (puberty, contraception and pregnancy) and hormonal treatments on the course of CU, including CSU and IU, in the presence of the women. RESULTS: Most of the women did not experience CU before puberty and if so, puberty did not influence the course of CU. Only 16 women had experienced a pregnancy during CU which caused a worsening of symptoms in four. Hormonal contraception was associated with aggravation in a minority of women, mostly women with CSU (10%). Women with isolated histaminic AE did not exhibit any female sex hormone dependency. CONCLUSIONS: It would appear that sex hormones act as a trigger in only a small subset of women with CU. Nevertheless, this should be taken into account to improve patient management.

The definition, diagnostic testing, and management of chronic inducible urticarias - The EAACI/GA(2) LEN/EDF/UNEV consensus recommendations 2016 update and revision.

These recommendations for the definition, diagnosis and management of chronic inducible urticaria (CIndU) extend, revise and update our previous consensus report on physical urticarias and cholinergic urticaria (Allergy, 2009). The aim of these recommendations is to improve the diagnosis and management of patients with CIndU. Our recommendations acknowledge the latest changes in our understanding of CIndU, and the available therapeutic options, as well as the development of novel diagnostic tools.

[Interest of allergy tests in urticaria]. / Place du bilan allergologique dans l'urticaire.

Urticaria is a common skin disease that may affect 20 % of the general population. Most of the time, urticaria is an acute disorder that rarely can be chronic. The difficulty in urticaria is not the clinical diagnosis because the rash is characteristic, but the underlying causes and treatment that result. Urticaria is a benign disease when chronic and potentially dangerous when acute and associated with allergy. This allergy risk, needs an allergy exploration, based on skin tests and / or specific IgE assays. Because allergy is unusual in chronic urticaria, no allergy tests should be performed. By contrast, these tests must be undertaken in case of acute urticaria with a strong suspicion of IgE-mediated reaction because of the risk of severe anaphylaxis in case of allergenic re-exposure.

The EAACI/GA(2) LEN/EDF/WAO Guideline for the definition, classification, diagnosis, and management of urticaria: the 2013 revision and update

This guideline is the result of a systematic literature review using the 'Grading of Recommendations Assessment, Development and Evaluation' (GRADE) methodology and a structured consensus conference held on 28 and 29 November 2012, in Berlin. It is a joint initiative of the Dermatology Section of the European Academy of Allergy and Clinical Immunology (EAACI), the EU-funded network of excellence, the Global Allergy and Asthma European Network (GA(2) LEN), the European Dermatology Forum (EDF), and the World Allergy Organization (WAO) with the participation of delegates of 21 national and international societies. Urticaria is a frequent, mast cell-driven disease, presenting with wheals, angioedema, or both. The life-time prevalence for acute urticaria is approximately 20%. Chronic spontaneous urticaria and other chronic forms of urticaria do not only cause a decrease in quality of life, but also affect performance at work and school and, as such, are members of the group of severe allergic diseases. This guideline covers the definition and classification of urticaria, taking into account the recent progress in identifying its causes, eliciting factors and pathomechanisms. In addition, it outlines evidence-based diagnostic and therapeutic approaches for the different subtypes of urticaria. This guideline was acknowledged and accepted by the European Union of Medical Specialists (UEMS).

Methods report on the development of the 2013 revision and update of the EAACI/GA2 LEN/EDF/WAO guideline for the definition, classification, diagnosis, and management of urticaria

This methods report describes the process of guideline development in detail. It is the result of a systematic literature review using the 'Grading of Recommendations Assessment, Development and Evaluation' (GRADE) methodology and a structured consensus conference held on 28 and 29 November 2012, in Berlin. It is a joint initiative of the Dermatology Section of the European Academy of Allergy and Clinical Immunology (EAACI), the EU-funded network of excellence, the Global Allergy and Asthma European Network (GA(2) LEN), the European Dermatology Forum (EDF), and the World Allergy Organization (WAO) with the participation of delegates of 21 national and international societies. This guideline covers the definition and classification of urticaria, taking into account the recent progress in identifying its causes, eliciting factors and pathomechanisms. In addition, it outlines evidence-based diagnostic and therapeutic approaches for the different subtypes of urticaria. This guideline was acknowledged and accepted by the European Union of Medical Specialists (UEMS) and is published in Allergy 2014; 69:868-887

Methods report on the development of the 2013 revision and update of the EAACI/GA2 LEN/EDF/WAO guideline for the definition, classification, diagnosis, and management of urticaria.

This methods report describes the process of guideline development in detail. It is the result of a systematic literature review using the 'Grading of Recommendations Assessment, Development and Evaluation' (GRADE) methodology and a structured consensus conference held on 28 and 29 November 2012, in Berlin. It is a joint initiative of the Dermatology Section of the European Academy of Allergy and Clinical Immunology (EAACI), the EU-funded network of excellence, the Global Allergy and Asthma European Network (GA(2) LEN), the European Dermatology Forum (EDF), and the World Allergy Organization (WAO) with the participation of delegates of 21 national and international societies. This guideline covers the definition and classification of urticaria, taking into account the recent progress in identifying its causes, eliciting factors and pathomechanisms. In addition, it outlines evidence-based diagnostic and therapeutic approaches for the different subtypes of urticaria. This guideline was acknowledged and accepted by the European Union of Medical Specialists (UEMS) and is published in Allergy 2014; 69:868-887.

The EAACI/GA(2) LEN/EDF/WAO Guideline for the definition, classification, diagnosis, and management of urticaria: the 2013 revision and update.

This guideline is the result of a systematic literature review using the 'Grading of Recommendations Assessment, Development and Evaluation' (GRADE) methodology and a structured consensus conference held on 28 and 29 November 2012, in Berlin. It is a joint initiative of the Dermatology Section of the European Academy of Allergy and Clinical Immunology (EAACI), the EU-funded network of excellence, the Global Allergy and Asthma European Network (GA(2) LEN), the European Dermatology Forum (EDF), and the World Allergy Organization (WAO) with the participation of delegates of 21 national and international societies. Urticaria is a frequent, mast cell-driven disease, presenting with wheals, angioedema, or both. The life-time prevalence for acute urticaria is approximately 20%. Chronic spontaneous urticaria and other chronic forms of urticaria do not only cause a decrease in quality of life, but also affect performance at work and school and, as such, are members of the group of severe allergic diseases. This guideline covers the definition and classification of urticaria, taking into account the recent progress in identifying its causes, eliciting factors and pathomechanisms. In addition, it outlines evidence-based diagnostic and therapeutic approaches for the different subtypes of urticaria. This guideline was acknowledged and accepted by the European Union of Medical Specialists (UEMS).

Comparison of CD63 Upregulation Induced by NSAIDs on Basophils and Monocytes in Patients with NSAID Hypersensitivity.

Background. An in vitro basophil activation test, based on the detection of CD63 upregulation induced by NSAIDs, has been described. Its clinical significance remains controversial. Objectives. In patients with a history of nonallergic NSAID hypersensitivity, stratified according to the severity of the symptoms, to assess with NSAIDs the predictive value of basophil (BAT) and monocyte (MAT) activation tests. Patients/Methods. Sixty patients who had NSAIDs-induced or exacerbated urticaria/angiooedema and 20 controls was included. After incubation with NSAIDs or acetaminophen, leukocytes were analysed for CD63 upregulation. Results. With aspirin, the sensitivity (37%) and specificity (90%) of BAT agree with already published results. In contrast, when patients had had cutaneous and visceral reactions, the frequency of positive BAT 14/22 (64%, P < 0.001) or MAT 10/22 (46%, P < 0.01) were increased. Conclusions. Positive tests were more frequent among patients having a severe hypersensitivity contrasting with the other patients who had results similar to controls.

[Management of drug reaction with eosinophilia and systemic symptoms (DRESS)]. / Prise en charge du drug reaction with eosinophilia and systemic symptoms (DRESS).

OBJECTIVE: The management of drug-induced hypersensitivity syndrome or drug reaction with eosinophilia and systemic symptoms (DRESS) is not codified. Demonstration of the reactivation of Herpesviruses illustrates the specific pathophysiology of this syndrome. Proposals for the management of DRESS were elaborated by the cutaneous adverse drug reaction working group of the French Society of Dermatology to help with its management. METHODS: From a review of literature and the experience of the members of this group, consensual proposals were written about diagnostic criteria, tests, treatment options, and follow-up. These proposals will need to be validated in prospective studies. RESULTS: A decisional tree of treatment options is proposed, based on the severity of visceral manifestations. The importance of a rapid withdrawal of the culprit drug and of a long-term follow-up is underlined. Treatment will be adapted to the clinicobiological status (topical corticosteroid, systemic corticosteroid, intravenous gammaglobulins, antivirals).

[Contact urticaria induced by hydrolyzed wheat proteins in cosmetics]. / Urticaire de contact aux hydrolysats de protéines de blé contenus dans des cosmétiques.

BACKGROUND: Hydrolyzed wheat protein, produced by hydrolysis of gluten, is used in certain cosmetics and foods as emulsifiers and stabilizers. It can induce contact urticaria to cosmetics and/or anaphylaxis to food via an immunologic mechanism. PATIENTS AND METHODS: A 28-year-old female beautician presented recurrent contact urticaria, initially on the hands and then more diffused, immediately after applying cosmetics of the same brand containing hydrolyzed wheat protein. Skin tests were positive with the cosmetics and with the hydrolyzed wheat protein contained therein. A 34-year-old woman presented four episodes of generalized urticaria after eating industrially prepared foods. She had also experienced contact urticaria with cosmetics. Skin tests with hydrolyzed wheat protein were positive. For both patients, withdrawal of cosmetics and foods containing hydrolyzed wheat protein led to the regression of symptoms. They were both tolerant to traditional wheat products, such as bread and pastries. DISCUSSION: Although contact urticaria to hydrolyzed wheat protein is rarely described, it must be understood since treatment by eradication of this product is simple and because contact urticaria may precede food allergy. Patients are tolerant to products containing unmodified wheat protein.

[Cheilitis: a new manifestation of gastro-oesophageal reflux?]. / Chéilite sèche : nouvelle manifestation du reflux gastro-oesophagien ?

BACKGROUND: Gastro-oesophageal reflux (GOR) is a common disease. Extra-oesophageal clinical signs associated with the disease and affecting the ENT (otitis media, rhinitis, laryngitis), pulmonary and stomatological spheres have been described for a number of years. We report three cases of cheilitis potentially attributable to GOR. CASE REPORT: Three female patients consulted for cheilitis present for several months. Screening for an infectious cause was negative, as was allergological investigation using skin tests. However, questioning revealed nocturnal breathing through the mouth in all three patients as well as symptomatic GOR in one patient. Test therapy comprising proton-pump inhibitors was proposed and after 1month symptoms had subsided completely. Gastro-oesophageal endoscopy performed in the subsequent weeks revealed oesophagitis in all patients. DISCUSSION: Extra-oesophageal signs described during the course of GOR mainly affect the ENT sphere and pulmonary sphere (cough, asthma) and are doubtless due to the direct caustic action of gastric secretions on the mucus membrane. Although gastroenterological investigation (endoscopy, 24-h pHmetry) may be negative, treatment with proton-pump inhibitors results in complete resolution of symptoms. The cases we describe suggest for the first time that extra-oesophageal sites affected by GOR may include the labial semimucosa.

The definition and diagnostic testing of physical and cholinergic urticarias--EAACI/GA2LEN/EDF/UNEV consensus panel recommendations.

The recommendations for the definition and diagnosis presented in this position paper are the result of a panel consensus meeting held in December 2008 in Berlin. This consensus meeting was a joint initiative of EAACI (European Academy of Allergology and Clinical Immunology) Dermatology Section, the EU-funded network of excellence, GA(2)LEN (Global Allergy and Asthma European Network), the EDF (European Dermatology Forum) and UNEV (urticaria network e.V.). The aim of these recommendations is to improve the diagnosis and management of patients with physical urticaria or cholinergic urticaria and to promote research and a better understanding of these diseases. Our recommendations used the paper produced by a 1996 expert meeting (1) and they acknowledge the latest changes in our understanding of physical urticarias and cholinergic urticaria as well as the recent development of novel diagnostic tools. In addition, this consensus paper highlights areas of need for further research.

[Safety of reintroducing platelet-inhibitory doses of aspirin in patients with urticaria or angioedema induced by anti-inflammatory doses]. / Réintroduction de l'aspirine à dose antiagrégante après urticaire ou angio-oedème induits par des doses anti-inflammatoires.

BACKGROUND: Aspirin is one of the most widely prescribed drugs in the world on account of its analgesic, antipyretic, and anti-inflammatory properties. Its effect on platelet aggregation makes it the first choice for prophylaxis in cardiovascular, neurological and obstetric diseases. However, a history of aspirin-induced urticaria and/or angioedema is usually a contraindication for further prescription of the drug. The aim of this article was to demonstrate that patients presenting aspirin-induced cutaneous reactions at anti-inflammatory doses can safely benefit from aspirin reintroduction at platelet-inhibitory doses. PATIENTS AND METHODS: Patients with a history of aspirin-induced urticaria and/or angioedema referred to our department between January 2000 and June 2008 for double-blind placebo-controlled reintroduction at platelet-inhibitory doses for a medical indication were enrolled in this study. RESULTS: Seventy patients with aspirin hypersensitivity as well as a medical indication for this therapy were referred to our department. Of these, 38 (54.3%) had a history of aspirin-induced urticaria and/or angioedema, including three laryngeal oedemas (7.9%). All subjects received platelet-inhibitory doses of aspirin (maximal total dose: 400mg/day) in double-blind placebo-controlled fashion during a one-day hospitalization period. None of the patients presented an immediate hypersensitivity reaction. Only one patient, who had received a cumulative dose of 200mg/day, reported diffuse urticaria and facial angioedema of no clinical significance the following day. He had a history of chronic urticaria. CONCLUSION: This article demonstrates the safety of reintroducing platelet-inhibitory doses of aspirin in patients in whom it is indicated and reporting aspirin-induced urticaria and/or angioedema with anti-inflammatory doses. However, patients with a history of chronic urticaria should undergo a challenge with the lowest platelet-inhibitory dose (75mg/day) in order to minimize the risk of aggravating their condition.

Physician and patient survey of allergic rhinitis in France: perceptions on prevalence, severity of symptoms, care management and specific immunotherapy.

BACKGROUND: Specific immunotherapy (SIT) is the only aetiological treatment used in allergic rhinitis (AR). A telephone survey of patients and physicians in France was carried out to understand better the real and perceived advantages and inconveniences of this therapeutic approach. METHODS: A cohort of 453 individuals with AR was selected using the Score For Allergic Rhinitis questionnaire. The survey evaluated the level of understanding of allergic rhinitis and its management, including both pharmacotherapy and SIT. A parallel survey was conducted with 400 general practitioners, allergists and nonallergist specialists. RESULTS: Approximately 50% of patients had heard about SIT as a therapeutic option. Of these, 56% had a positive view of SIT and 14% a negative image. A majority of patients and physicians with a positive opinion associated SIT with improved well-being and quality of life, while those with a negative opinion considered it to be a long and inconvenient treatment, with uncertain results. Over 50% of patients who had been offered SIT had accepted it and approximately 60% of these were satisfied with it. The future availability of SIT as sublingual tablets was perceived positively by both patients and physicians. CONCLUSIONS: Many patients with AR are unaware of their pathology and few seek help from health professionals. When patients take medication, they are generally satisfied with their treatment, even if it is only symptomatic. Patients and physicians see the notion of definitive recovery as the main benefit of SIT, whereas the main disadvantage is the duration of treatment.