Orticarie da freddo acquisite e familiari

Translated title of the contribution: Acquired and familial cold urticarias

Nicoletta Cassano, Michelangelo Vestita, Gino Antonio Vena

Research output: Contribution to journalArticlepeer-review


Cold urticaria is triggered by the exposure of skin or mucous membranes to cold, but it actually corresponds to a heterogeneous group of different clinical entities. In fact, cold urticaria can be distinguished into acquired, either primary or secondary, and familiar forms, as well as into typical and atypical variants. Acquired cold urticaria (ACU) is properly a physical urticaria and has been associated with various diseases, including infections and cryopathies. Pathogenesis of ACU is still unknown, although the release of histamine and other proinflammatory mediators is likely to have a crucial role. Immunoglobulins and even autoantibodies responsible for mast cell degranulation have been implicated. Familial cold urticaria should be regarded as a hereditary autoinflammatory syndrome ("familial cold autoinflammatory syndrome" - FCAS). FCAS is a rare syndrome based on an autosomal dominant condition, which represents the mildest phenotype in the spectrum of cryopyrin-associated periodic syndromes that are associated with mutations in a common gene: CIAS-1. This gene encodes NALP3 (also known as cryopyrin), an important mediator of inflammation and interleukin 1beta processing. Diagnosis of cold urticaria is based on history and clinical findings and should be confirmed by cold challenge in typical forms. In atypical and familiar forms, cold can trigger the reaction only in the presence of particular cofactors. For the management of an ACU patient it is essential to avoid eliciting stimuli and prophlylactic treatment when cold exposure can not be avoided. First-line treatment approach consists in the administration of new generation H1-antihistamines which can be administered at higher dosages than those recommended to increase effectiveness. Treatment of the underlying disease is mandatory in secondary forms of ACU. Induction of tolerance may be tried very cautiously in selected refractory cases. Some reports documented the positive therapeutic results obtained with the use of systemic antibiotics (e.g., penicillin or tetracyclines) and, very recently, of omalizumab. Preliminary evidences support the potential usefulness of the recombinant interleukin-1 receptor antagonist anakinra in the treatment of FCAS.

Translated title of the contributionAcquired and familial cold urticarias
Original languageItalian
Pages (from-to)1-9
Number of pages9
JournalAnnali Italiani di Dermatologia Allergologica Clinica e Sperimentale
Issue number1
Publication statusPublished - Jan 2009

ASJC Scopus subject areas

  • Dermatology
  • Immunology and Allergy


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