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Cold urticaria

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

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Cold urticaria

  • acquired cold urticaria (ACU) is a subtype of physical urticaria that is caused by the release of proinflammatory mast cell mediators after cold exposure
  • ACU
    • characterized by the development of weal-and-flare type skin reactions and/or angiooedema caused by release of histamine, leukotrienes and other proinflammatory mast-cell mediators after exposure of the skin to cold (1)
    • symptoms typically occur minutes after the skin is exposed to cold air, liquids or objects - usually limited to cold-exposed skin areas
      • note though that extensive cold contact may result in generalized urticarial symptoms and/or in systemic reactions including headache, dyspnoea, hypotension and loss of consciousness - most frequently results from extensive cold contact during water exposure
        • patients with a history of oropharyngeal oedema seem to be at particularly high risk for developing shock-like reactions after aquatic activity
    • most frequently affects young adults - affects women more than men (2:1)
      • mean duration of the disease is 4-5 years (1)
        • remission or at least improvement of symptoms in 50% of patients within 5 years
        • incidence of ACU has been estimated to be 0.05%
          • higher incidences are found in regions with a cold climate
  • differential diagnosis
    • subdivided into primary and secondary ACU - are different designations in accordance with an unknown (primary) or suspected (secondary) underlying cause or disease for ACU
      • ACU can be the secondary manifestation of underlying hematologic or infectious diseases (eg, cryoglobulinemia or mononucleosis)
      • there are also some very rare atypical subtypes of cold urticaria
        • includes two hereditary familial cold syndromes: delayed cold urticaria and familial cold auto-inflammatory syndrome (FCAS)
  • aetiology
    • causes and mechanisms involved in the aetiology and pathogenesis largely unknown
      • reported associations with viral or bacterial infections including borreliosis, hepatitis, infectious mononucleosis, and human immunodeficiency virus infection
        • other associations include with Helicobacter pylori colonization, acute toxoplasmosis and other parasitic infections
        • infections of the upper respiratory tract, teeth or urogenital tract may also be associated with ACU
        • infrequent immunological findings in patients with ACU include cryoglobulinaemia, composed of monoclonal IgG and mixed types of IgG/IgM and IgG/IgA/cryoglobulins
        • prevalence of functional anti-IgE antibodies (IgG and IgM) has been described
        • an association with haematological, lymphatic or neoplastic diseases has been reported
  • diagnosis/investigation
    • requires specialist review
      • cold-provocation testing -a positive immediate cold-stimulation test (CST), i.e. the development of urticarial skin lesions at sites of cold challenge, verifies the presence of ACU
  • management
    • seek specialist advice
    • avoidance of cold - avoidance of cold exposure is desirable (but not always achievable)
    • symptomatic therapy
      • antihistamine treatment is the most common and the most effective symptomatic therapeutic option to prevent and reduce patients reactions after cold exposure - however, sufficient reduction of urticarial symptoms in many patients with ACU requires high dosing with antihistamines, up to four times the daily recommended dose
      • other treatment options for the therapy of severe ACU with high risk of life-threatening reactions and/or an insufficient response to antihistamines, include the concomitant use of leucotriene antagonists, ciclosporin, corticosteroids
      • there is study evidence that combination therapy with antihistamines and leukotriene receptor antagonists is more effective than each drug given alone (2)
    • curative therapy
      • antibiotic therapy should be considered in some patients (1)
        • occasionally, patients with ACU have been shown to benefit from such treatment even if no underlying infection can be detected
    • further treatment options
      • induction of cold tolerance (hardening) is an effective method of treating patients with ACU
      • treatment with topical capsaicin has been reported to prevent ACU symptoms

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