COLD URTICARIA

By Gary M. White, MD

Cold urticaria

Urticarial lesions at the site of ice cube contact x several minutes.


Cold urticaria (CU) is the development of urticaria in skin exposed to cold air, surfaces or fluids.

Clinical

An urticarial wheal develops in skin exposed to cold, e.g. cold water or air. The throat or lips may swell after ingesting cold foods. Rarely, a patient has drowned after swimming in cold water, presumably from the systemic urticarial reaction. Infusion of cold intravenous solutions pose a similar risk.

Differential Diagnosis

The presence of cryoglobulins and cryofibrinogens and cold aglutinins should all be excluded. Rarely, cold urticaria may be secondary to a B cell malignancy [JEADV 2016;30;2066].

The ice cube test is diagnostic and is performed as follows. A plastic bag containing ice (so as not to induce aquagenic urticaria) is held against the skin of the volar forearm for 5 minutes, and the contact site evaluated for whealing in 10 minutes.

Familial cold autoinflammatory syndrome (FCAS), formerly known as familial cold urticaria, is a rare condition characterized by fever, rash, and arthralgias elicited by exposure to cold.

A new syndrome, PLAID (PLCG2-associated antibody deficiency and immune dysregulation) is characterized by cold urticaria, autoimmunity, atopy and humoral immune deficiency, result in recurrent sinopulmonary infections [JAMA Derm 2015;151;627].

Treatment

The patient should be warned about the risks of anaphylaxis.   Specific mention of the dangers of swimming or bathing in cold water should be discussed.

An epinephrine auto injector may be prescribed.   Non-sedating antihistamines should be recommended but often results are unsatisfactory.  Up to 4 times the standard dosage should be recommended. At these doses, sedation may be a problem so a gradual increase of the dose should be recommended.   The combination of cetirizine 10 mg/day and zafirlukast 20 mg BID was more effective than either agent alone in one patient.  Mizolastine (10 mg, once daily) was shown to be superior to placebo for both delaying and reducing the cold-induced wheal reaction without significant adverse events. Omalizumab may be used. Case reports of benefit with prednisone and cyclosporin have been reported. Cold desensitization may be tried, but extreme caution is necessary for those with known systemic reactions.

Additional Pictures

Cold urticaria

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