Highlights & Basics
- Urticaria is a skin condition characterized by erythematous, blanching, edematous, nonpainful, pruritic lesions that typically resolve within 24 hours and leave no residual markings.
- Acute urticaria lasts less than 6 weeks and is often due to a hypersensitivity reaction to a specific trigger. Underlying viral infections are also a common cause of acute urticaria, particularly in children. Acute urticaria is generally self-limited.
- Chronic urticaria is characterized by daily or near-daily episodes of hives occurring for 6 weeks or more and has a complex etiology.
- Angioedema is swelling involving the deeper layers of the subdermis and occurs in association with urticaria in about 40% of cases. It can also occur in the absence of urticaria.
- Angioedema involving the face or neck can potentially compromise the airway and requires prompt airway management.
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Definition
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Citations
Zuberbier T, Abdul Latiff AH, Abuzakouk M, et al. The international EAACI/GA²LEN/EuroGuiDerm/APAAACI guideline for the definition, classification, diagnosis, and management of urticaria. Allergy. 2022 Mar;77(3):734-66.[Abstract][Full Text]
Fine LM, Bernstein JA. Guideline of chronic urticaria beyond. Allergy Asthma Immunol Res. 2016 Sep;8(5):396-403.[Abstract][Full Text]
Powell RJ, Leech SC, Till S, et al. BSACI guideline for the management of chronic urticaria and angioedema. Clin Exp Allergy. 2015;45:547-565.[Abstract][Full Text]
Maurer M, Magerl M, Betschel S, et al. The international WAO/EAACI guideline for the management of hereditary angioedema-The 2021 revision and update. Allergy. 2022 Jul;77(7):1961-90.[Abstract][Full Text]
1. Zuberbier T, Abdul Latiff AH, Abuzakouk M, et al. The international EAACI/GA²LEN/EuroGuiDerm/APAAACI guideline for the definition, classification, diagnosis, and management of urticaria. Allergy. 2022 Mar;77(3):734-66.[Abstract][Full Text]
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3. Greaves MW. Chronic urticaria. N Engl J Med. 1995;332:1767-1772.[Abstract]
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6. Fine LM, Bernstein JA. Guideline of chronic urticaria beyond. Allergy Asthma Immunol Res. 2016 Sep;8(5):396-403.[Abstract][Full Text]
7. Kaplan AP. Urticaria and angioedema. In: Middleton's allergy: principles and practice, vol 2. 6th ed. Philadelphia, PA: Mosby; 2003:1537-1558.
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33. Powell RJ, Leech SC, Till S, et al. BSACI guideline for the management of chronic urticaria and angioedema. Clin Exp Allergy. 2015;45:547-565.[Abstract][Full Text]
34. Tosoni C, Lodi-Rizzini F, Cinquini M, et al. A reassessment of diagnostic criteria and treatment of idiopathic urticarial vasculitis: a retrospective study of 47 patients. Clin Exp Dermatol. 2009 Mar;34(2):166-70.[Abstract][Full Text]
35. Bernstein JA, Lang DM, Khan DA, et al. The diagnosis and management of acute and chronic urticaria: 2014 update. J Allergy Clin Immunol. 2014 May;133(5):1270-7.[Abstract][Full Text]
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38. Maurer M, Magerl M, Betschel S, et al. The international WAO/EAACI guideline for the management of hereditary angioedema-The 2021 revision and update. Allergy. 2022 Jul;77(7):1961-90.[Abstract][Full Text]
39. Kaplan AP. Urticaria and angioedema. In: Adkinson NF, Bochner B, Busse W, et al, eds. Middleton's allergy: principles and practice, vol 2. 7th ed. Philadelphia, PA: Mosby, 2009;1063-1081.
40. Georgy MS, Pongracic JA. Chapter 22: Hereditary and acquired angioedema. Allergy Asthma Proc. 2012 May-Jun;33 Suppl 1:73-76.[Abstract][Full Text]
41. Barniol C, Dehours E, Mallet J, et al. Levocetirizine and prednisone are not superior to levocetirizine alone for the treatment of acute urticaria: a randomized double-blind clinical trial. Ann Emerg Med. 2018 Jan;71(1):125-31.e1.[Abstract][Full Text]
42. Shertzer CL, Lookingbill DP. The effects of relaxation therapy and hypnotizability in chronic urticaria. Arch Dermatol. 1987;123:197-201.[Abstract]
43. Handa S. Comparative efficacy of cetirizine and fexofenadine in the treatment of chronic idiopathic urticaria. J Derm Treat. 2004;15:55-57.[Abstract]
44. Simons FE, Simons KJ. H1 antihistamines: current status and future directions. World Allergy Organ J. 2008 Sep;1(9):145-55.[Abstract][Full Text]
45. Fein MN, Fischer DA, O'Keefe AW, et al. CSACI position statement: Newer generation H1-antihistamines are safer than first-generation H1-antihistamines and should be the first-line antihistamines for the treatment of allergic rhinitis and urticaria. Allergy Asthma Clin Immunol. 2019;15:61.[Abstract][Full Text]
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48. Asero R. Chronic unremitting urticaria: is the use of antihistamines above the licensed dose effective? A preliminary study of cetirizine at licensed and above-licensed doses. Clin Exp Derm. 2006;32:34-38.[Abstract]
49. Sharma M, Bennett C, Cohen SN, et al. H1-antihistamines for chronic spontaneous urticaria. Cochrane Database Syst Rev. 2014 Nov 14;(11):CD006137.[Abstract][Full Text]
50. Brunet C, Bedard PM, Hebert J. Effects of H1-antihistamine drug regimen on the histamine release by nonlesional skin mast cells of patients with chronic urticaria. J Allergy Clin Immunol. 1990;86:787-793.[Abstract]
51. Goldsobel AB, Rohr AS, Siegel SC, et al. Efficacy of doxepin in the treatment of chronic idiopathic urticaria. J Allergy Clin Immunol. 1986;78:867-873.[Abstract]
52. Monroe EW, Cohen SH, Kalbfleisch J, et al. Combined H1 and H2 antihistamine therapy in chronic urticaria. Arch Dermatol. 1981;117:404-407.[Abstract]
53. Harvey RP, Wegs J, Schocket AL. A controlled trial of therapy in chronic urticaria. J Allergy Clin Immunol. 1981;68:262-266.[Abstract]
54. Ellis MH. Successful treatment of chronic urticaria with leukotriene antagonists. J Allergy Clin Immunol. 1998;102:876-877.[Abstract]
55. Spector S, Tan RA. Antileukotrienes in chronic urticaria. J Allergy Clin Immunol. 1998;101:572.[Abstract]
56. Reimers A, Pichler C, Helbling A, et al. Zafirlukast has no beneficial effects in the treatment of chronic urticaria. Clin Exp Allergy. 2002;32:1763-1768.[Abstract]
57. Di Lorenzo G, Pacor ML, Mansueto P, et al. Randomized placebo-controlled trial comparing desloratadine and montelukast in monotherapy and desloratadine plus montelukast in combined therapy for chronic urticaria. J Allergy Clin Immunol. 2004;114:619-625.[Abstract]
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59. Bagenstose SE, Levin L, Bernstein JA. The addition of zafirlukast to cetirizine improves the treatment of chronic urticaria in patients with positive autologous serum skin test results. J Allergy Clin Immunol. 2004;113:134-140.[Abstract]
60. Perez C, Sanchez-Borges M, Capriles E, et al. Pretreatment with montelukast blocks NSAID-induced urticaria and angioedema. J Allergy Clin Immunol. 2001;108:1060-1061.[Abstract]
61. Food and Drug Administration Drug Safety Communication. FDA requires Boxed Warning about serious mental health side effects for asthma and allergy drug montelukast (Singulair); advises restricting use for allergic rhinitis. Mar 2020 [internet publication].[Full Text]
62. Maurer M, Rosén K, Hsieh HJ, et al. Omalizumab for the treatment of chronic idiopathic or spontaneous urticaria. N Engl J Med. 2013;368:924-935.[Abstract]
63. Saini S, Rosen KE, Hsieh HJ, et al. A randomized, placebo-controlled, dose-ranging study of single-dose omalizumab in patients with H1-antihistamine-refractory chronic idiopathic urticaria. J Allergy Clin Immunol. 2011;128:567-573.[Abstract]
64. Tharp MD, Bernstein JA, Kavati A, et al. Benefits and harms of omalizumab treatment in adolescent and adult patients with chronic idiopathic (spontaneous) urticaria: a meta-analysis of "real-world" evidence. JAMA Dermatol. 2019 Jan;155(1):29-38.[Abstract][Full Text]
65. Maurer M, Metz M, Brehler R, et al. Omalizumab treatment in patients with chronic inducible urticaria: a systematic review of published evidence. J Allergy Clin Immunol. 2018 Feb;141(2):638-49.[Abstract][Full Text]
66. Harrison CA, Bastan R, Peirce MJ, et al. Role of calcineurin in the regulation of human lung mast cell and basophil function by cyclosporine and FK506. Br J Pharmacol. 2007 Feb;150(4):509-18.[Abstract][Full Text]
67. Grattan CE, O'Donnell BF, Francis DM, et al. Randomized double-blind study of cyclosporine in chronic 'idiopathic' urticaria. Br J Dermatol. 2000;143:365-372.[Abstract]
68. Vena GA, Cassano N, Colombo D, et al. Cyclosporine in chronic idiopathic urticaria, a double-blind, randomized, placebo-controlled trial. J Am Acad Dermatol. 2006;55:705-709.[Abstract]
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71. Zuraw BL, Busse PJ, White M, et al. Nanofiltered C1 inhibitor concentrate for treatment of hereditary angioedema. NEJM 2010;363:513-522.[Abstract][Full Text]
72. Bork K, Meng G, Staubach P. Treatment with C1 inhibitor concentrate in abdominal pain attacks of patients with hereditary angioedema. Transfusion 2005;45:1774-1784.[Abstract]
73. Cicardi M, Levy RJ, McNeil DL, et al. Ecallantide for the treatment of acute attacks in hereditary angioedema. NEJM 2010;363:523-531.[Abstract][Full Text]
74. Cicardi M, Banerji A, Bracho F, et al. Icatibant, a new bradykinin-receptor antagonist, in hereditary angioedema. N Engl J Med. 2010 Aug 5;363(6):532-41.[Abstract][Full Text]
75. Zuraw BL, Bernstein JA, Lang DM, et al. A focused parameter update: hereditary angioedema, acquired C1 inhibitor deficiency, and angiotensin-converting enzyme inhibitor-associated angioedema. J Allergy Clin Immunol. 2013 Jun;131(6):1491-3.[Abstract][Full Text]
76. Bork K, Hardt J, Staubach-Renz P, et al. Risk of laryngeal edema and facial swellings after tooth extraction in patients with hereditary angioedema with and without prophylaxis with C1 inhibitor concentrate: a retrospective study. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2011 Jul;112(1):58-64.[Abstract]
77. Martinez-Saguer I, Rusicke E, Aygören-Pürsün E, et al. Pharmacokinetic analysis of human plasma-derived pasteurized C1-inhibitor concentrate in adults and children with hereditary angioedema: a prospective study. Transfusion. 2010 Feb;50(2):354-60.[Abstract]
78. Longhurst H, Zinser E. Prophylactic therapy for hereditary angioedema. Immunol Allergy Clin North Am. 2017 Aug;37(3):557-70.[Abstract]
79. Longhurst H, Cicardi M, Craig T, et al. Prevention of hereditary angioedema attacks with a subcutaneous C1 inhibitor. N Engl J Med. 2017 Mar 23;376(12):1131-40.[Abstract][Full Text]
80. Bork K, Bygum A, Hardt J. Benefits and risks of danazol in hereditary angioedema: a long-term survey of 118 patients. Ann Allergy Asthma Immunol. 2008 Feb;100(2):153-61.[Abstract]
81. Kaplan A, Ferrer M, Bernstein JA, et al. Timing and duration of omalizumab response in patients with chronic idiopathic/spontaneous urticaria. J Allergy Clin Immunol. 2016;137:474-481.[Abstract][Full Text]
82. Zhao ZT, Ji CM, Yu WJ, et al. Omalizumab for the treatment of chronic spontaneous urticaria: a meta-analysis of randomized clinical trials. J Allergy Clin Immunol. 2016;137:1742-1750.[Abstract]
83. Manning ME, Kashkin JM. Berotralstat (BCX7353) is a novel oral prophylactic treatment for hereditary angioedema: Review of phase II and III studies. Allergy Asthma Proc. 2021 Jul 14;42(4):274-82.[Abstract][Full Text]
84. Zuraw B, Lumry WR, Johnston DT, et al. Oral once-daily berotralstat for the prevention of hereditary angioedema attacks: A randomized, double-blind, placebo-controlled phase 3 trial. J Allergy Clin Immunol. 2021 Jul;148(1):164-172.e9.[Abstract][Full Text]
85. National Institute for Health and Care Excellence. Berotralstat for preventing recurrent attacks of hereditary angioedema. Oct 2021 [internet publication].[Full Text]
86. Banerji A, Riedl MA, Bernstein JA, et al. Effect of lanadelumab compared with placebo on prevention of hereditary angioedema attacks: a randomized clinical trial. JAMA. 2018 Nov 27;320(20):2108-21. [Erratum in: JAMA. 2019 Apr 23;321(16):1636.][Abstract][Full Text]
87. Riedl MA, Bernstein JA, Craig T, et al. An open-label study to evaluate the long-term safety and efficacy of lanadelumab for prevention of attacks in hereditary angioedema: design of the HELP study extension. Clin Transl Allergy. 2017;7:36.[Abstract][Full Text]
88. ClinicalTrials.gov. A study of STAR-0215 in healthy adult participants. ClinicalTrials.gov identifier: NCT05477160. Jan 2023 [internet publication].[Full Text]
89. ClinicalTrials.gov. A study of STAR-0215 in participants with hereditary angioedema. ClinicalTrials.gov identifier: NCT05695248. Aug 2023 [internet publication].[Full Text]
90. Maurer M, Giménez-Arnau AM, Sussman G, et al. Ligelizumab for chronic spontaneous urticaria. N Engl J Med. 2019 Oct 3;381(14):1321-32.[Abstract][Full Text]
91. Caballero T, Farkas H, Bouillet L, et al. International consensus and practical guidelines on the gynecologic and obstetric management of female patients with hereditary angioedema caused by C1 inhibitor deficiency. J Allergy Clin Immunol. 2012 Feb;129(2):308-20.[Abstract][Full Text]
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