Cold urticaria, or cold contact urticaria, is a subtype of physical urticaria (table 1) [1,2]. The physical urticarias are forms of inducible urticaria, in which there is a specific stimulus that triggers the development of symptoms. Cold urticaria is characterized by pruritic wheals (hives) and/or angioedema due to cutaneous mast cell activation and release of proinflammatory mediators after cold exposure. Triggers include skin contact with cold objects, cold liquids, and cold air. The underlying pathophysiology is largely unknown.
Cold urticaria is reviewed here. Other urticaria disorders, including acute and chronic spontaneous urticaria and other forms of inducible urticarial including physical urticaria, are discussed separately. (See "Physical urticarias" and "New onset urticaria" and "Chronic urticaria: Standard management and patient education" and "Chronic urticaria: Clinical manifestations, diagnosis, pathogenesis, and natural history".)
Cold urticaria most frequently affects young adults [3,4]. Both sexes are affected with similar frequency in most studies [3,5,6], although one study reported that females were affected twice as often as males . Up to one-half of patients with cold urticaria are atopic and one-fourth have other types of inducible urticaria, most commonly symptomatic dermographism and cholinergic urticaria [5,7].
The incidence of cold urticaria was estimated to be 0.05 percent in Central Europe . The frequency of cold urticaria among physical urticaria subtypes varies between 5 and 34 percent, partly depending upon the geographic region (ie, higher incidences are found in regions with colder climates and lower rates are seen in regions with a warmer climate) [3,5,8,9].
Natural history — Cold urticaria is a self-limited disorder in many patients. Remission, or at least improvement of symptoms, occurs in 50 percent of patients within five to six years [2-5,10]. The mean time to resolution in one study was 5.6 ± 3.5 years . Recurrence of cold urticaria after remission is rare. However, some patients have the disorder for many years and there are variant forms that appear to be lifelong (eg, familial atypical cold urticaria). (See 'Variant forms' below.)