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Review
. 2024 Aug;44(3):421-438.
doi: 10.1016/j.iac.2024.03.002. Epub 2024 May 18.

Chronic Spontaneous Urticaria: Etiology and Pathogenesis

Eric T Oliver  1 , Sarbjit S Saini  2
Affiliations
Review

Chronic Spontaneous Urticaria: Etiology and Pathogenesis

Eric T Oliver et al. Immunol Allergy Clin North Am. 2024 Aug.

Abstract

Urticaria, also known as hives, is a common condition thought to affect up to 20% of individuals worldwide in their lifetime. This skin condition is characterized by the appearance of pruritic, erythematous papules or plaques with superficial swelling of the dermis. The major complaint is the symptom of pruritus. Angioedema, which involves a deeper swelling of dermal or mucosal tissues, may accompany urticaria. Urticaria can be classified by both time course of symptoms and the underlying etiology.

Keywords: Basophils; Chronic urticaria; Eosinophils; Mast cells.

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Conflict of interest statement

Disclosure E.T. Oliver: Grant Support: National Institutes of Health, United States (K23AI139394), Robert Meyerhoff Professorship Advisory Board/Consultant: Novartis, Regeneron/Sanofi. S.S. Saini: Grant Support: Novartis, Switzerland, Sanofi, France, Regeneron, United States, National Institutes of Health, United States, Allakos, Escient Advisory Board/Consultant: Allakos, Granular Therapeutics, Novartis, Aquestive, Regeneron, Escient, Evommune, Innate, Celltrion, Sanofi.

Figures

Fig. 1.
Fig. 1.
(A) Image of skin lesion. Superficial swellings of the dermis are called wheals. (B) Biopsy of of early spontaneous wheal shows perivascular lymphocytes and intraluminal and perivascular neutrophils, with perivascular and interstitial eosinophils. (From Grattan CE, Sabroe RA, Greaves MW. J Am Acad Dermatol 2002;46(5):645–60. Available at: http://dx.doi.org/10.1067/mjd.2002.122759; with permission).
Fig. 2.
Fig. 2.
Proposed pathogenic antibodies in CSU. TPO, thyroperoxidase; Ig, immunoglobulin; MC, mast cell. (From Kocatürk et al. Viruses. 2023 Jul 20;15(7):1585. doi: 10.3390/v15071585. PMID: 37515272; PMCID: PMC10386070)
Fig. 3.
Fig. 3.
Timeline for autoantibody theory. (Data from Refs,,,–)
Fig. 4.
Fig. 4.
An autologous serum skin test (ASST) reaction. Phosphate-buffered saline (P.B.S.), saline solution negative control, serum, and plasma are injected in a volume of 0.05 mL and the reaction read at 30 minutes. Here, both serum and plasma have produced positive responses. (From Greaves M. Chronic urticaria. J Allergy Clin Immunol 2000;105(4):664–72; with permission).
Fig. 5.
Fig. 5.
Basophil assays. Approach for measuring histamine release or activation markers. BAT, basophil activation test (Adapted from Brodell LA, Beck LA, Saini SS. Pathophysiology of chronic urticaria. Ann Allergy Asthma Immunol 2008;100(4):294; with permission)
Fig. 6.
Fig. 6.
Urticaria. (Courtesy of New Zealand Dermatological Society Incorporated).
Fig. 7.
Fig. 7.
Urticaria in skin of color. (Courtesy of New Zealand Dermatological Society Incorporated).
Fig. 8.
Fig. 8.
Angioedema. Deeper swelling of the skin and submucosa called angioedema are less well demarcated and do not necessarily show any difference in color from unaffected skin. (From Grattan CE, Sabroe RA, Greaves MW. Chronic urticaria. J Am Acad Dermatol 2002;46(5):645–60; with permission).
See this image and copyright information in PMC

References

    1. Kolkhir P, Gimenez-Arnau AM, Kulthanan K, Peter J, Metz M, Maurer M. Urticaria. Nat Rev Dis Primers. 2022;8(1):61-z. doi: 10.1038/s41572-022-00389-z. - DOI - PubMed
    1. Ornek Ozdemir S, Kuteyla Can P, Degirmentepe EN, Cure K, Singer R, Kocaturk E . A comparative analysis of chronic inducible urticaria in 423 patients: Clinical and laboratory features and comorbid conditions. J Eur Acad Dermatol Venereol. 2023. doi: 10.1111/jdv.19637. - DOI - PubMed
    1. Buttgereit T, Vera C, Aulenbacher F, et al. Patients with chronic spontaneous urticaria who have wheals, angioedema, or both, differ demographically, clinically, and in response to treatment-results from CURE. J Allergy Clin Immunol Pract. 2023;11(11):3515–3525.e4. doi: 10.1016/j.jaip.2023.08.020. - DOI - PubMed
    1. Sabate-Bresco M, Rodriguez-Garijo N, Azofra J, et al. A comparative study of sex distribution, autoimmunity, blood, and inflammatory parameters in chronic spontaneous urticaria with angioedema and chronic histaminergic angioedema. J Allergy Clin Immunol Pract. 2021;9(6):2284–2292. doi: 10.1016/j.jaip.2021.03.038. - DOI - PubMed
    1. Gaig P, Olona M, Munoz Lejarazu D, et al. Epidemiology of urticaria in spain. J Investig Allergol Clin Immunol. 2004;14(3):214–220. - PubMed

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