Definition and research guide
This page combines a MeSH definition, publication statistics and selected research abstracts. It is a starting point for reading the literature, not a medical textbook or a systematic evidence review.
Content available: 3 complete abstracts (669 words), 9 references. Full article text is not stored or reproduced here; follow the PMID, DOI or PMC links to read the original sources and their access terms.
Definition
A vascular reaction of the skin characterized by erythema and wheal formation due to localized increase of vascular permeability. The causative mechanism may be allergy, infection, or stress.
Definition: NLM Medical Subject Headings (MeSH) scope note (Public domain (U.S. National Library of Medicine); attribution requested). View the MeSH record.
Also known as: Urticarias; Hives; Urticarial Wheals; Urticarial Wheal; Wheals, Urticarial; Wheal, Urticarial.
Where it fits in the MeSH classification
- Broader topics
- Skin Diseases, Vascular; Hypersensitivity, Immediate
- Narrower topics
- Angioedema; Chronic Urticaria; Cold Urticaria
Research landscape
PubMed indexes 20,439 records tagged with the MeSH heading “Urticaria” (counts retrieved 2026-10-29). 3,692 of them (18.1%) were published from 2020 onward, and the busiest period so far is through 1999 with 8,035 records.
Reviews account for 3,199 records (15.7%) and meta-analyses for 134. Randomized controlled trials number 585 (2.9%) as a share of all records on the topic.
Show the data
| Period | Records |
|---|---|
| through 1999 | 8,035 |
| 2000-2009 | 3,456 |
| 2010-2019 | 5,256 |
| 2020 onward | 3,692 |
Publication-type chart data
| Type | Records | Share |
|---|---|---|
| Reviews | 3,199 | 15.7% |
| Meta-analyses | 134 | 0.7% |
| Randomized controlled trials | 585 | 2.9% |
Method: PubMed query "Urticaria"[MeSH Terms] with publication-type and date filters, retrieved 2026-10-29.
Reading the evidence in immunology
Immune responses are studied in model organisms, in cells from donors and in patients, and the same pathway can behave differently across these settings. Mouse immune systems differ from human ones in important ways, and blood measurements may not reflect what happens in tissues. Clinical conclusions about immune-mediated disease or immunotherapy come mainly from controlled trials and long-term cohorts. Check the species and tissue studied, whether markers were linked to outcomes patients notice, and the follow-up time.
Evidence from open-access studies
These open-access studies come from PubMed’s best-match results for this heading (papers whose main topic it is), ranked here by citation count, with one recent addition. Each abstract is reproduced in full under its recorded open licence so that you can read the authors’ own words. They illustrate the literature; they are not a systematic summary of it. Citation counts measure attention, not reliability or clinical benefit.
| Study / authors | Year | Source label / design | Europe PMC citations | Licence / source |
|---|---|---|---|---|
| Mechanisms of NLRP3 activation and inhibition elucidated by functional analysis of disease-associated variants | 2025 | Research article Design not recorded in structured metadata | 84 | cc by PMID 39930093 · DOI · PMC11876074 |
| Hereditary Angioedema with Normal C1 Inhibitor: an Updated International Consensus Paper on Diagnosis, Pathophysiology, and Treatment | 2025 | Review article Design not recorded in structured metadata | 49 | cc by PMID 40053270 · DOI · PMC11889046 |
| The International Guideline for the Definition, Classification, Diagnosis and Management of Urticaria | 2026 | Review article Design not recorded in structured metadata | 47 | cc by PMID 41649409 · DOI · PMC13466004 |
Mechanisms of NLRP3 activation and inhibition elucidated by functional analysis of disease-associated variants [1]
Abstract reading summary — source excerpts
- Opening excerpt
- The NLRP3 inflammasome is a multiprotein complex that mediates caspase-1 activation and the release of proinflammatory cytokines, including interleukin (IL)-1β and IL-18.
- Closing excerpt
- Overall, our study provides improved diagnosis for patients with CAPS, mechanistic insight into the activation of NLRP3 and stratification of patients for the future application of targeted therapeutics.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (280 words)
The NLRP3 inflammasome is a multiprotein complex that mediates caspase-1 activation and the release of proinflammatory cytokines, including interleukin (IL)-1β and IL-18. Gain-of-function variants in the gene encoding NLRP3 (also called cryopyrin) lead to constitutive inflammasome activation and excessive IL-1β production in cryopyrin-associated periodic syndromes (CAPS). Here we present functional screening and automated analysis of 534 NLRP3 variants from the international INFEVERS registry and the ClinVar database. This resource captures the effect of NLRP3 variants on ASC speck formation spontaneously, at low temperature, after inflammasome stimulation and with the specific NLRP3 inhibitor MCC950. Most notably, our analysis facilitated the updated classification of NLRP3 variants in INFEVERS. Structural analysis suggested multiple mechanisms by which CAPS variants activate NLRP3, including enhanced ATP binding, stabilizing the active NLRP3 conformation, destabilizing the inactive NLRP3 complex and promoting oligomerization of the pyrin domain. Furthermore, we identified pathogenic variants that can hypersensitize the activation of NLRP3 in response to nigericin and cold temperature exposure. We also found that most CAPS-related NLRP3 variants can be inhibited by MCC950; however, NLRP3 variants with changes to proline affecting helices near the inhibitor binding site are resistant to MCC950, as are variants in the pyrin domain, which likely trigger activation directly with the pyrin domain of ASC. Our findings could help stratify the CAPS population for NLRP3 inhibitor clinical trials and our automated methodologies can be implemented for molecules with a different mechanism of activation and in laboratories worldwide that are interested in adding new functionally validated NLRP3 variants to the resource. Overall, our study provides improved diagnosis for patients with CAPS, mechanistic insight into the activation of NLRP3 and stratification of patients for the future application of targeted therapeutics.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 39930093 · DOI · PMC11876074
Hereditary Angioedema with Normal C1 Inhibitor: an Updated International Consensus Paper on Diagnosis, Pathophysiology, and Treatment [2]
Abstract reading summary — source excerpts
- Opening excerpt
- Hereditary angioedema (HAE) has been recognized for almost 150 years.
- Closing excerpt
- This review and expert opinion on the best practice approach to diagnosing and treating HAE-nC1INH will support physicians to better manage patients with HAE-nC1INH.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (203 words)
Hereditary angioedema (HAE) has been recognized for almost 150 years. The newest form of HAE, where C1 inhibitor levels are normal (HAE-nC1INH), was first described in 2000. Over the last two decades, new types of apparent non-mast cell-mediated angioedema with normal quantity and activity of C1INH have been described, in some cases with proven genetic pathogenic variants that co-segregate with angioedema expression within families. Like HAE due to C1INH deficiency, HAE-nC1INH patients are at risk of serious morbidity and mortality. Therefore, proactive management and treatment of HAE-nC1INH patients after an expert physician diagnosis is critically important. The underlying pathophysiology responsible for the angioedema has also been clarified in some of the HAE-nC1INH types. While several clinical guidelines and practice parameters including HAE-nC1INH have been published, we have made substantial progress in our understanding encompassing diagnostic criteria, pathophysiology, and treatment outcomes. HAE International (HAEi) and the US HAE Association (HAEA) convened a symposium of global HAE-nC1INH experts to synthesize our current knowledge in the area. Given the paucity of high-level evidence in HAE-nC1INH, all recommendations are based on expert opinion. This review and expert opinion on the best practice approach to diagnosing and treating HAE-nC1INH will support physicians to better manage patients with HAE-nC1INH.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 40053270 · DOI · PMC11889046
The International Guideline for the Definition, Classification, Diagnosis and Management of Urticaria [3]
Abstract reading summary — source excerpts
- Opening excerpt
- This update and revision of the international guideline for urticaria was developed in accordance with the methods recommended by Cochrane and the Grading of Recommendations Assessment, Development and Evaluation (GRADE) working group.
- Closing excerpt
- 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.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (186 words)
This update and revision of the international guideline for urticaria was developed in accordance with the methods recommended by Cochrane and the Grading of Recommendations Assessment, Development and Evaluation (GRADE) working group. It is an initiative of the Global Allergy and Asthma Excellence Network (GA2LEN) and its Urticaria and Angioedema Centers of Reference and Excellence (UCAREs and ACAREs), with the participation of 210 delegates from 107 national and international societies, from 59 countries. The consensus conference was held on December 6th, 2024. This guideline was acknowledged and accepted by the European Union of Medical Specialists (UEMS). Urticaria is a frequent, mast cell-driven disease, defined by a rapid appearance of wheals, angioedema, or both. The lifetime prevalence of acute urticaria is estimated to be approximately 20%. Chronic urticaria, categorized as either chronic spontaneous urticaria or chronic inducible urticaria, is disabling, impairs quality of life, and affects performance at work and school, however, novel therapies are available. 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.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 41649409 · DOI · PMC13466004
Key reviews and meta-analyses
The most cited review-type papers among PubMed’s best-match results for this heading, listed with bibliographic details only. Follow the links for the full text and access terms.
- Research article The international WAO/EAACI guideline for the management of hereditary angioedema-The 2021 revision and update [4]
- Research article Prevalence of chronic urticaria in children and adults across the globe: Systematic review with meta-analysis [5]
- Research article Urticaria [6]
- Research article Autoimmune chronic spontaneous urticaria [7]
- Research article Updosing nonsedating antihistamines in patients with chronic spontaneous urticaria: a systematic review and meta-analysis [8]
- Research article Chronic spontaneous urticaria: Focus on pathophysiology to unlock treatment advances [9]
References
- Feng S, Wierzbowski MC, Hrovat-Schaale K, Dumortier A, Zhang Y, Zyulina M, et al.. Mechanisms of NLRP3 activation and inhibition elucidated by functional analysis of disease-associated variants. . 2025. PMID 39930093 · DOI · PMC11876074
- Zuraw BL, Bork K, Bouillet L, Christiansen SC, Farkas H, Germenis AE, et al.. Hereditary Angioedema with Normal C1 Inhibitor: an Updated International Consensus Paper on Diagnosis, Pathophysiology, and Treatment. . 2025. PMID 40053270 · DOI · PMC11889046
- Zuberbier T, Ansari ZA, Abdul Latiff AH, Abuzakouk MM, Agcaoili-De Jesus MS, Agondi RC, et al.. The International Guideline for the Definition, Classification, Diagnosis and Management of Urticaria. . 2026. PMID 41649409 · DOI · PMC13466004
- Maurer M, Magerl M, Betschel S, Aberer W, Ansotegui IJ, Aygören-Pürsün E, et al.. The international WAO/EAACI guideline for the management of hereditary angioedema-The 2021 revision and update. Allergy. 2022. PMID 35006617 · DOI
- Fricke J, Ávila G, Keller T, Weller K, Lau S, Maurer M, et al.. Prevalence of chronic urticaria in children and adults across the globe: Systematic review with meta-analysis. Allergy. 2020. PMID 31494963 · DOI
- Kolkhir P, Giménez-Arnau AM, Kulthanan K, Peter J, Metz M, Maurer M. Urticaria. Nat Rev Dis Primers. 2022. PMID 36109590 · DOI
- Kolkhir P, Muñoz M, Asero R, Ferrer M, Kocatürk E, Metz M, et al.. Autoimmune chronic spontaneous urticaria. J Allergy Clin Immunol. 2022. PMID 35667749 · DOI
- Guillén-Aguinaga S, Jáuregui Presa I, Aguinaga-Ontoso E, Guillén-Grima F, Ferrer M. Updosing nonsedating antihistamines in patients with chronic spontaneous urticaria: a systematic review and meta-analysis. Br J Dermatol. 2016. PMID 27237730 · DOI
- Kaplan A, Lebwohl M, Giménez-Arnau AM, Hide M, Armstrong AW, Maurer M. Chronic spontaneous urticaria: Focus on pathophysiology to unlock treatment advances. Allergy. 2023. PMID 36448493 · DOI
About this article
- Sources
- NLM Medical Subject Headings (definition, synonyms); NCBI PubMed (counts, identifiers); Europe PMC (open-access abstracts and citation counts).
- Verification
- Every reference was resolved against PubMed on 2026-10-31; title, year and DOI matched.
- Review state
- automated source verified; no human expert review has been recorded yet.
- Version
- 1, last updated 2026-10-29.
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Evidence strength
EvidenceHelix's computed view of the study designs behind this topic: how common meta-analyses, randomized trials and reviews are, how recent the literature is, and a plain-language reading. It describes the design mix of the literature. It is not a quality appraisal of any study, and it is not medical advice.