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Diseases

Genetic and Rare Diseases Information Center (GARD)

Muckle-Wells syndrome


Other Names for this Disease
  • Muckle Wells syndrome
  • Neutrophilic urticaria
  • UDA syndrome
  • Urticaria, deafness and amyloidosis
  • Urticaria-deafness-amyloidosis syndrome
Related Diseases
  • Secondary glomerular disease
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Overview

Muckle-Wells syndrome is an autoinflammatory disease, and the intermediate form of cryopyrin-associated periodic syndrome (CAPS). Signs and symptoms may include recurrent episodes of fever, skin rash, joint pain, abdominal pain, and pinkeye; progressive sensorineural deafness; and amyloidosis. It is caused by mutations in the NLRP3 gene and is inherited in an autosomal dominant manner. Treatment includes a medication called Anakinra during acute episodes.[1]
Last updated: 4/7/2014

References

  1. Delwyn Dyall-Smith. Muckle-Wells syndrome. DermNet NZ. December 29, 2013; http://dermnetnz.org/systemic/muckle-wells.html. Accessed 4/7/2014.
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Basic Information

  • The Cleveland Clinic provides an overview of the different types of periodic fever syndromes.
  • DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.
  • Genetics Home Reference (GHR) contains information on Muckle-Wells syndrome. This website is maintained by the National Library of Medicine.
  • The United States Food and Drug Administration approved a new drug for the treatment of rare inflammatory syndromes, including Muckle-Wells syndrome, in February of 2008. More information about Muckle-Wells syndrome and this treatment can be found by clicking on the above link.
  • The National Organization for Rare Disorders (NORD) is a federation of more than 130 nonprofit voluntary health organizations serving people with rare disorders. Click on the link to view information on this topic.

In Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs.  Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Muckle-Wells syndrome. Click on the link to view a sample search on this topic.

Selected Full-Text Journal Articles

Other Names for this Disease
  • Muckle Wells syndrome
  • Neutrophilic urticaria
  • UDA syndrome
  • Urticaria, deafness and amyloidosis
  • Urticaria-deafness-amyloidosis syndrome
Related Diseases
  • Secondary glomerular disease
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.