ArticleCase reports in dermatology
Recurrent Urticaria: A Rare Cryopyrin-Associated Periodic Syndrome - Muckle-Wells Syndrome.
Article in Case reports in dermatology. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
2 citing papers in PubMed.
- Mechanisms outpacing medicine in autoinflammatory diseases.Frontiers in immunology · 2026Review
- Review
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Authors and funding
2 authors.
Funding
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Abstract
Introduction: Muckle-Wells syndrome (MWS) is a rare cryopyrin-associated periodic syndrome (CAPS), an autoinflammatory disorder due to a loss of function mutation in the NACHT domain of the NLRP3 gene. The loss of cryopyrin activity brought on by this deficiency eventually causes dysregulated inflammation and increased release of the proinflammatory cytokine interleukin (IL)-1 beta. It has an autosomal dominant inheritance. Case Presentation: We present here an 8-year-old girl with recurrent fever, recurrent urticarial rash, sensorineural hearing loss, raised inflammatory markers and serum amyloid levels, which did not respond to the anti-histaminic drugs, was wrongly diagnosed as tuberculosis, which on further genetic evaluation was diagnosed as MWS. Conclusion: Despite being uncommon, treating clinicians should take MWS into consideration given the specific clinical presentation. With the availability of appropriate medications, future complications can be prevented, and the prognosis is better.
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