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Familial cold urticaria

Orpha number ORPHA47045
Synonym(s) FCAS
FCAS1
FCU
Familial cold autoinflammatory syndrome
Prevalence 1-9 / 1 000 000
Inheritance
  • Autosomal dominant
Age of onset Neonatal/infancy
ICD-10
  • L50.2
OMIM
UMLS
  • C0343068
MeSH -
MedDRA
  • 10064570
SNOMED CT
  • 238687000

Summary

Familial cold urticaria (FCAS) is the mildest form of cryopyrin-associated periodic syndrome (CAPS; see this term) and is characterized by recurrent episodes of urticaria triggered by exposure to cold associated with low-grade fever, and arthralgia.


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Detailed information

Review article
  • EN (2005,pdf)
Clinical practice guidelines
  • FR (2013,pdf)
Guidance for genetic testing
  • EN (2012,pdf)
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