Chronic spontaneous urticaria

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Grattan, C.; Hebert, J.; Hide, M.; Kaplan, A.; Kapp, A.; Katelaris, C. H.; Kocatürk, E.; Kulthanan, K.; Larenas-Linnemann, D.; Leslie, T. A.; Magerl, M.; Mathelier-Fusade, P.; Meshkova, R. Y.; Metz, M.; Nast, A.; Nettis, E.; Oude-Elberink, H.; Rosumeck, S.; Saini, S. S.; Sánchez-Borges, M.; Schmid-Grendelmeier, P.; Staubach, P.; Sussman, G.; Toubi, E.; Vena, G. A.; Vestergaard, C.; Wedi, B.; Werner, R. N.; Zhao, Z.; Maurer, M. (June 25, 2018).

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Sánchez-Borges, M.; Schmid-Grendelmeier, P.; Simons, F. E. R.; Staubach, P.; Sussman, G.; Toubi, E.; Vena, G. A.; Wedi, B.; Zhu, X. J.; Maurer, M. (April 30, 2014). "The <scp>EAACI</scp>/<scp>GA</scp><scp>LEN</scp>/<scp>EDF</scp>/<scp>WAO</scp> Guideline for the definition, classification, diagnosis, and management of urticaria: the 2013 revision and update".

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Schoepke, Nicole; Asero, Riccardo; Ellrich, André; Ferrer, Marta; Gimenez-Arnau, Ana; E. H. Grattan, Clive; Jakob, Thilo; Konstantinou, George N.; Raap, Ulrike; Skov, Per Stahl; Staubach, Petra; Kromminga, Arno; Zhang, Ke; Bindslev-Jensen, Carsten; Daschner, Alvaro; Kinaciyan, Tamar; Knol, Edward F.;

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Metz, Martin; Altrichter, Sabine; Buttgereit, Thomas; Fluhr, Joachim W.; Fok, Jie Shen; Hawro, Tomasz; Jiao, Qingqing; Kolkhir, Pavel; Krause, Karoline; Magerl, Markus; Pyatilova, Polina; Siebenhaar, Frank; Su, Huichun; Terhorst-Molawi, Dorothea; Weller, Karsten; Xiang, Yi-Kui; Maurer, Marcus (2021).

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In various areas of the world, the standard workup is different. A very comprehensive history is something that is universally agreed upon. The main goal is to identify any urticaria-inducing factors, as the most straightforward course of treatment is to eliminate them, including physical provocation

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According to recent data, there are three subgroups of CSU: autoimmunity type I (CSUaiTI, also known as "autoallergic CSU"), autoimmunity type IIb (CSUaiTIIb), and CSU with an unidentified cause (CSUuc). Type I and type IIb autoimmunity may coexist in some cases. The underlying pathomechanism in the

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The standard workup for CSU differs in different parts of the world. However, most doctors agree on the importance of having a detailed history. The main goal is to identify any urticaria-inducing factors because eliminating them is the most straightforward course of treatment. Basic laboratory

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Bossi, F.; Frossi, B.; Radillo, O.; Cugno, M.; Tedeschi, A.; Riboldi, P.; Asero, R.; Tedesco, F.; Pucillo, C. (2011). "Mast cells are critically involved in serum-mediated vascular leakage in chronic urticaria beyond high-affinity IgE receptor stimulation: Mast cells and vascular leakage in chronic

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Zuberbier, T.; Aberer, W.; Asero, R.; Abdul Latiff, A. H.; Baker, D.; Ballmer-Weber, B.; Bernstein, J. A.; Bindslev-Jensen, C.; Brzoza, Z.; Buense Bedrikow, R.; Canonica, G. W.; Church, M. K.; Craig, T.; Danilycheva, I. V.; Dressler, C.; Ensina, L. F.; Giménez-Arnau, A.; Godse, K.; Gonçalo, M.;

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Zuberbier, T.; Aberer, W.; Asero, R.; Bindslev-Jensen, C.; Brzoza, Z.; Canonica, G. W.; Church, M. K.; Ensina, L. F.; Giménez-Arnau, A.; Godse, K.; Gonçalo, M.; Grattan, C.; Hebert, J.; Hide, M.; Kaplan, A.; Kapp, A.; Abdul Latiff, A. H.; Mathelier-Fusade, P.; Metz, M.; Nast, A.; Saini, S. S.;

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in CSUaiTIIb. Other mechanisms that are currently unknown have significance for the degranulation of skin mast cells in CSUuc. Furthermore, modulating factors like medications, stress, or infections can change how sensitive skin mast cells are to degranulators, which can lead to increased

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should be part of the diagnostic process if UV as well as an autoinflammatory disease is suspected. This is so that neutrophilic infiltrates or vascular destruction can be checked for. It could be challenging to differentiate UV from CSU because there are currently no established standardized

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of any kind. Similar figures were discovered in a recent Spanish study. Nonetheless, studies conducted in Europe suggest a lower lifetime prevalence, or the prevalence observed throughout one's lifetime up until the investigation, of approximately 8–10%. There is less information on nonacute

796:, must be taken into consideration in patients presenting with frequent angioedema without wheals. Here, the doctor should closely examine the patient's history, age at symptom onset, duration of attacks, presence of abdominal angioedema episodes, use of concurrent medications (particularly 486:

The majority of patients with chronic spontaneous urticaria frequently linked multiple triggers to flare-ups. However, the suspected trigger does not always result in symptoms, so patients frequently subject themselves to needless limitations and lifestyle modifications.

697:-controlled food provocation, pressure, heat, cold, and others should be used if an eliciting factor is suspected in order to confirm if it is an eliciting factor. Because chronic as well as recurrent infections are known to cause urticaria, only differential blood counts and 661:. These factors were not present in the skin that is not affected. The pathophysiology of chronic urticaria is the subject of several theories, none of which has been proven beyond a reasonable doubt. Research has looked at the validity of serologic testing to establish an 1660:

Maurer, M.; Weller, K.; Bindslev-Jensen, C.; Giménez-Arnau, A.; Bousquet, P. J.; Bousquet, J.; Canonica, G. W.; Church, M. K.; Godse, K. V.; Grattan, C. E. H.; Greaves, M. W.; Hide, M.; Kalogeromitros, D.; Kaplan, A. P.; Saini, S. S.; Zhu, X. J.; Zuberbier, T. (2011).

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The majority of guidelines discourage food as the cause of chronic urticaria; nonetheless, patients frequently believe that certain foods aggravate their condition or are the cause of it. Between 13 and 80% of people self-report that food triggers their CSU episodes.

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According to one study examining the course of urticaria in the general population, 50% of patients with chronic urticaria had no symptoms after three months, and 80% had no symptoms after twelve months. Still, 11% experienced urticaria after five years.

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is the second, and its goal is to relieve symptoms. Although removing the cause is the ideal course of action, this may not be feasible in many situations. According to current guidelines, a therapeutic approach should be implemented in three steps: (1) taking a

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concentration, and function in every patient with frequent angioedema in whom hereditary angioedema as well as an acquired C1 inhibitor deficiency cannot be ruled out in order to rule out or confirm hereditary angioedema due to C1 inhibitor deficiency.

752:(CBC) with differential, are crucial to detect signs of systemic inflammation and rule out autoinflammatory conditions as well as UV with systemic involvement. However, these results can also be influenced by other comorbidities and can be seen in CSU. 3042:

Kulthanan, Kanokvalai; Chaweekulrat, Pichanee; Komoltri, Chulaluk; Hunnangkul, Saowalak; Tuchinda, Papapit; Chularojanamontri, Leena; Maurer, Marcus (2018). "Cyclosporine for Chronic Spontaneous Urticaria: A Meta-Analysis and Systematic Review".

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Kay, A. B.; Ying, S.; Ardelean, E.; Mlynek, A.; Kita, H.; Clark, P.; Maurer, M. (2014). "Calcitonin gene-related peptide and vascular endothelial growth factor are expressed in lesional but not uninvolved skin in chronic spontaneous urticaria".

720:, in addition to itchy wheals or angioedema, in order to rule out both conditions. Extended periods of time exceeding twenty-four hours and a gradual resolution of individual wheals indicate UV exposure; further indications of systemic 910:

urticaria. A study conducted forty years ago in Sweden found a point prevalence of about 0.1% in the population overall, and a different study conducted in Spain more recently reported a point prevalence of 0.6% in the population.

313:. The hives and angioedema seen in CSU is thought to be linked to the degranulation of skin mast cells. Mast cells release proteases, histamine, cytokines, and arachidonic acid metabolites, causing swelling, redness, and itching. 399:, these lesions may appear flattened and take on a range of sizes. It can affect any part of the body, including parts where clothing might press against the skin. Lesions typically do not last more than 24 hours. The degree of 1517:

Trevisonno, Jordan; Balram, Bhairavi; Netchiporouk, Elena; Ben-Shoshan, Moshe (May 10, 2015). "Physical urticaria: Review on classification, triggers and management with special focus on prevalence including a meta-analysis".

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is not advised because the side effects increase with dosage and duration and eventually result in greater disability than CSU. However, until other treatments take effect, acute symptoms can be managed with a brief course of

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Sánchez, Jorge; Amaya, Emerson; Acevedo, Ana; Celis, Ana; Caraballo, Domingo; Cardona, Ricardo (2017). "Prevalence of Inducible Urticaria in Patients with Chronic Spontaneous Urticaria: Associated Risk Factors".

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For the treatment of chronic spontaneous urticaria, a two-pronged strategy has been proposed. The underlying cause(s) and/or eliciting trigger(s) must first be identified and eliminated. The second approach is

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Kozel, Martina M.A.; Bossuyt, Patrick M.M.; Mekkes, Jan R.; Bos, Jan D. (2003). "Laboratory tests and identified diagnoses in patients with physical and chronic urticaria and angioedema: A systematic review".

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Bonnekoh, H.; Scheffel, J.; Maurer, M.; Krause, K. (November 28, 2017). "Use of skin biomarker profiles to distinguish Schnitzler syndrome from chronic spontaneous urticaria: results of a pilot study".

384:, or both can be signs of chronic spontaneous urticaria. Between 40 and 50 percent of CSU patients experience angioedema. However, angioedema is the main symptom reported by about 10% of patients. 1474:

Confino-Cohen, Ronit; Chodick, Gabriel; Shalev, Varda; Leshno, Moshe; Kimhi, Oded; Goldberg, Arnon (2012). "Chronic urticaria and autoimmunity: Associations found in a large population study".

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Maurer, Marcus; Costa, Celia; Gimenez Arnau, AnaMaria; Guillet, Gerard; Labrador-Horrillo, Moises; Lapeere, Hilde; Meshkova, Raisa; Savic, Sinisa; Chapman-Rothe, Nadine (September 3, 2020).

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A contributing factor to the exacerbation of chronic spontaneous urticaria in certain patients may be stress. On the other hand, urticaria is most likely one of the main sources of stress.

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A two-pronged strategy has been proposed for the treatment of chronic spontaneous urticaria. First, the underlying cause(s) and/or eliciting trigger(s) must be established and eliminated.

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lesions to long-lasting plaque-like lesions, even though they may also present with urticarial lesions. Patients with coexisting wheals and plaques and who are pregnant are said to have

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Gaig, P; Olona, M; Muñoz Lejarazu, D; Caballero, M T; Domínguez, F J; Echechipia, S; García Abujeta, J L; Gonzalo, M A; Lleonart, R; Martínez Cócera, C; Rodríguez, A; Ferrer, M (2004).

1886:"TH1/TH2 cytokines and inflammatory cells in skin biopsy specimens from patients with chronic idiopathic urticaria: Comparison with the allergen-induced late-phase cutaneous reaction" 430:. Often affected body parts are the lips, eyes, cheeks, and limbs. Urticaria and angioedema typically coexist, but in a small percentage of cases, angioedema may be the only symptom. 1935:"Elevations in T-helper-2-initiating cytokines (interleukin-33, interleukin-25 and thymic stromal lymphopoietin) in lesional skin from chronic spontaneous ('idiopathic') urticaria" 2220:

JACQUES, P; LAVOIE, A; BEDARD, P; BRUNET, C; HEBERT, J (1992). "Chronic idiopathic urticaria: Profiles of skin mast cell histamine release during active disease and remission".

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Peroni, Anna; Colato, Chiara; Schena, Donatella; Girolomoni, Giampiero (2010). "Urticarial lesions: If not urticaria, what else? The differential diagnosis of urticaria".

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Peroni, Anna; Colato, Chiara; Zanoni, Giovanna; Girolomoni, Giampiero (2010). "Urticarial lesions: If not urticaria, what else? The differential diagnosis of urticaria".

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Zuberbier, T.; Balke, M.; Worm, M.; Edenharter, G.; Maurer, M. (April 26, 2010). "Epidemiology of urticaria: a representative cross-sectional population survey".

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Sabroe, Ruth A.; Poon, Eric; Orchard, Guy E.; Lane, David; Francis, David M.; Barr, Robert M.; Black, Martin M.; Black, Anne Kobza; Greaves, Malcolm W. (1999).

716:. Doctors should ask about the duration as well as resolution of each wheal as well as the presence of any other signs and symptoms, such as fever episodes or 478:

had higher rates of these conditions than those with chronic urticaria in a study involving a database of 13,000 patients compared to 10,000 control subjects.

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Kaplan, Allen P.; Joseph, Kusumam; Saini, Sarbjit S. (2015). "How omalizumab came to be studied as a therapy for chronic spontaneous/idiopathic urticaria".

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While the cause of chronic spontaneous urticaria is unknown many infividuals with chronic urticaria have been found to have a higher prevalence of various

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There is evidence that individuals with chronic urticaria are more likely to have a variety of autoimmune diseases. Researchers found that patients with

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Wu, Maddalena Alessandra; Perego, Francesca; Zanichelli, Andrea; Cicardi, Marco (2016). "Angioedema Phenotypes: Disease Expression and Classification".

302:, or both for more than six weeks. The most common symptoms of chronic spontaneous urticaria are angioedema and hives that are acompanied by itchiness. 1837:"Cutaneous inflammatory cell infiltrate in chronic idiopathic urticaria: Comparison of patients with and without anti-FcϵRI or anti-IgE autoantibodies" 3589: 2952:"IgM and IgA in addition to IgG autoantibodies against FcɛRIα are frequent and associated with disease markers of chronic spontaneous urticaria" 349:, which aims to alleviate symptoms. A therapeutic approach should be implemented in three steps, according to current guidelines: (1) taking a 3132:

Sheldon, John M.; Mathews, Kenneth P.; Lovell, Robert G. (1954). "The vexing urticaria problem: Present concepts of etiology and management".

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once daily; (2) increasing the daily dose of the second-generation antihistamine up to four times; and (3) pursuing off-label therapy with

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Makris, Michael; Marrouche, Nadine; Schmid-Grendelmeier, Peter; Sussman, Gordon; Toubi, Elias; Church, Martin K.; Maurer, Marcus (2019).

3880: 442:. Many patients with chronic spontaneous urticaria report that certain triggers, like stress, infections, certain foods, or 3754: 2263:

Nettis, E.; Dambra, P.; Loria, M. P.; Cenci, L.; Vena, G. A.; Ferrannini, A.; Tursi, A. (2001). "Mast-cell phenotype in urticaria".

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It has been discovered by American authors that approximately one in five individuals will at some point in their lives suffer from

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Omalizumab works well even in the most difficult, resistant situations. Despite having nearly as good of an efficacy as omalizumab,

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Kolkhir, Pavel; Muñoz, Melba; Asero, Riccardo; Ferrer, Marta; Kocatürk, Emek; Metz, Martin; Xiang, Yi-Kui; Maurer, Marcus (2022).

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Schmetzer, Oliver; Lakin, Elisa; Topal, Fatih A.; Preusse, Patricia; Freier, Denise; Church, Martin K.; Maurer, Marcus (2018).

1663:"Unmet clinical needs in chronic spontaneous urticaria. A GA2LEN task force report1: Unmet clinical needs in chronic urticaria" 213: 2801:"Correlations between disease activity, autoimmunity and biological parameters in patients with chronic spontaneous urticaria" 494:. The second most common physical trigger that was reported was pressure. The third most commonly reported trigger was cold. 3449: 1788:"Studies of the cellular infiltrate of chronic idiopathic urticaria: Prominence of T-lymphocytes, monocytes, and mast cells" 755:

Patients with recurrent wheals need to have a number of other conditions taken into consideration. Certain conditions, like

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theory of illness. Other theories include additional serologic factors, abnormalities of tissue mast cells, and basophils.

2173:"Detection of Low-Molecular-Weight Mast Cell–Activating Factors in Serum From Patients With Chronic Spontaneous Urticaria" 3784: 1329:

Zingale, L. C.; Beltrami, L.; Zanichelli, A.; Maggioni, L.; Pappalardo, E.; Cicardi, B.; Cicardi, M. (October 24, 2006).

443: 310: 1618:"Dietary Habits in Patients with Chronic Spontaneous Urticaria: Evaluation of Food as Trigger of Symptoms Exacerbation" 658: 3987: 205: 3397:(6). The Korean Academy of Asthma, Allergy and Clinical Immunology and The Korean Academy of Pediatric Al: 326–331. 2950:

Altrichter, Sabine; Zampeli, Vasiliki; Ellrich, André; Zhang, Ke; Church, Martin K; Maurer, Marcus (June 18, 2020).

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Kaplan, Allen; Lebwohl, Mark; Giménez-Arnau, Ana M.; Hide, Michihiro; Armstrong, April W.; Maurer, Marcus (2023).

2851:"Biomarkers and clinical characteristics of autoimmune chronic spontaneous urticaria: Results of the PURIST Study" 1702:

KAPLAN, A; HORAKOVA, Z; KATZ, S (1978). "Assessment of tissue fluid histamine levels in patients with urticaria".

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Chronic spontaneous urticaria is defined by the presence of wheals, angioedema, or both for more than six weeks.

654: 350: 3663: 745: 702: 322: 168: 1156:"Antihistamine-resistant chronic spontaneous urticaria remains undertreated: 2-year data from the AWARE study" 1097:"The EAACI/GA²LEN/EDF/WAO guideline for the definition, classification, diagnosis and management of urticaria" 1042:

Termeer, Christian; Staubach, Petra; Kurzen, Hjalmar; Strömer, Klaus; Ostendorf, Rolf; Maurer, Marcus (2015).

3937: 3898: 924: 782: 201: 2086:

Kolkhir, Pavel; Church, Martin K.; Weller, Karsten; Metz, Martin; Schmetzer, Oliver; Maurer, Marcus (2017).

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autoantibodies directed against mast cell receptors that are activated cause mast cell activation and

646: 455: 3942: 3918: 3863: 3724: 756: 550: 209: 1044:"Chronic spontaneous urticaria – a management pathway for patients with chronic spontaneous urticaria" 987:"Chronic spontaneous urticaria: Clinical manifestations, diagnosis, pathogenesis, and natural history" 3962: 3853: 3848: 725: 334: 605:. This infiltrate bears resemblance to the infiltrate observed in the allergen late-phase reaction. 305:

Chronic spontaneous urticaria, despite its cause being unknown, is linked to a higher prevalence of

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Bakke, P.; Gulsvik, A.; Eide, G. E. (1990). "Hay fever, eczema and urticaria in southwest Norway".

771:(also called polymorphic eruption in pregnancy). A skin biopsy is necessary to confirm premonitory 490:

In one study, the most common type of idiopathic urticaria among CSU patients was symptomatic

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de Montjoye, L.; Darrigade, A.S.; Gimenez Arnau, A.; Herman, A.; Dumoutier, L.; Baeck, M. (2021).

2044:"IL-24 is a common and specific autoantigen of IgE in patients with chronic spontaneous urticaria" 879:

is regarded as third line because it carries a much higher risk of side effects. Long-term use of

3913: 3656: 2850: 793: 809: 180: 712:(UV) are uncommon but should be taken into consideration in patients who experience recurrent 3812: 3729: 3673: 3481: 3444: 789: 709: 562: 338: 185: 142: 90: 2901:"Co-occurrence of IgE and IgG autoantibodies in patients with chronic spontaneous urticaria" 3957: 3807: 3764: 3759: 3714: 919: 749: 738: 729: 717: 459: 326: 160: 2899:

Asero, R; Marzano, A V; Ferrucci, S; Lorini, M; Carbonelli, V; Cugno, M (March 17, 2020).

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Brodell, Lindsey A.; Beck, Lisa A. (2008). "Differential diagnosis of chronic urticaria".

8: 3903: 3835: 3779: 3734: 3506: 2414: 2412: 2410: 2408: 197: 3092: 2473:"Chronic spontaneous urticaria: latest developments in aetiology, diagnosis and therapy" 1314: 1281: 763:, are uncommon. The primary skin lesions in these patients vary, ranging from permanent 471: 3774: 3769: 3719: 3639: 3421: 3386: 3310: 3290: 3259: 3208: 3188: 2935: 2900: 2881: 2781: 2687: 2505: 2472: 2464: 2453: 2340: 2307: 2288: 2153: 2024: 1972: 1551: 1363: 1330: 1252: 1219: 772: 764: 741: 698: 690: 439: 318: 306: 164: 61: 3511: 3334:"Chronic spontaneous urticaria: Focus on pathophysiology to unlock treatment advances" 3145: 2577: 2405: 2171:

Cugno, M; Tedeschi, A; Frossi, B; Bossi, F; Marzano, AV; Asero, R (October 19, 2016).

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HELLGREN, LARS (1972). "The Prevalence of Urticaria in the Total Population".

2761: 2323: 1467: 1297: 1268: 557:. These mediators cause swelling, redness, and itching by stimulating sensory 3981: 3840: 3412: 3369: 3298: 3247: 3196: 3153: 3064: 3020: 2977: 2926: 2877: 2826: 2769: 2726: 2675: 2631: 2585: 2550: 2496: 2488: 2441: 2390: 2331: 2284: 2241: 2213: 2198: 2113: 2069: 2012: 1960: 1926: 1911: 1862: 1813: 1772: 1723: 1643: 1594: 1539: 1495: 1452: 1411: 1354: 1305: 1243: 1191: 1132: 1069: 839: 801: 797: 785: 614: 610: 558: 530: 491: 396: 254: 225: 3464: 1902: 1885: 1884:

Ying, Sun; Kikuchi, Yoko; Meng, Qiu; Kay, A.Barry; Kaplan, Allen P. (2002).

1384: 1382: 509: 3947: 3869: 3430: 3377: 3255: 3161: 3111: 3072: 3028: 2985: 2834: 2777: 2734: 2683: 2639: 2593: 2514: 2449: 2398: 2349: 2206: 2121: 2020: 1968: 1919: 1870: 1688: 1634: 1617: 1602: 1547: 1503: 1460: 1372: 1261: 1199: 1182: 1140: 1077: 944: 876: 813: 721: 682: 662: 566: 330: 246: 98: 3306: 3204: 2249: 1821: 1015:"Chronic spontaneous urticaria: Standard management and patient education" 128:, heat, tight clothing or straps, stress, variations in diet, and alcohol. 3567: 1731: 1403: 1379: 865: 733: 518: 415: 362: 172: 133: 54: 2381: 2364: 2189: 2172: 1659: 1418: 1346: 3799: 3360: 3041: 2798: 2081: 2079: 1516: 1123: 869: 778: 737:

histopathologic criteria for UV. Basic laboratory tests, which include

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Borriello, Francesco; Granata, Francescopaolo; Marone, Gianni (2014).

2004: 1951: 1934: 1172: 1155: 1113: 1096: 1060: 1043: 329:(CBC) with differential, are critical for detecting signs of systemic 3604: 3528: 2792: 906: 574: 542: 514: 407: 357:

daily dose up to four times; and (3) pursuing off-label therapy with

262: 66: 2076: 1695: 1567: 1565: 1089: 1087: 3929: 3826: 2527: 1616:

Sánchez, Jorge; Sánchez, Andres; Cardona, Ricardo (June 19, 2018).

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degranulation, and a perivascular infiltrate of cells, including

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Journal of Investigational Allergology & Clinical Immunology

3090: 1747:"Mast cell number and phenotype in chronic idiopathic urticaria" 1473: 1093: 3491: 3476: 3168: 2646: 2605: 2603: 2128: 1983: 1153: 713: 606: 602: 534: 295: 125: 2943: 2177:

Journal of Investigational Allergology and Clinical Immunology

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is evident in the lesion cytokine profile, indicating a mixed

2949: 2418: 1933:

Kay, A.B.; Clark, P.; Maurer, M.; Ying, S. (April 12, 2015).

1609: 808:, and prodromal symptoms. Laboratory evaluation must involve 638: 618: 570: 427: 411: 388: 381: 189: 3225: 2892: 2704: 2652: 2609: 2600: 2521: 2356: 1041: 775:

in the elderly when there is no sign of vesicles or bullae.

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