ABSTRACT. Objective. To characterize the features of cold urticaria in children, with particular focus on systemic reactions, because little pediatric data are available.Methodology. Chart reviews of 30 children <18 years old who were evaluated in the past 3 years at the Children's Hospital Allergy Program (Boston, MA) and a private allergy practice. Demographic, diagnostic, and therapeutic data were collected. Telephone interviews of patients and/or their parents were performed to obtain follow-up data.Results. Our data showed that the mean and median ages of onset were ϳ7 years. No secondary causes were found. One third of patients had anaphylactic reactions. These reactions could not be predicted based on available variables. Patients with negative cold-stimulation test (ice-cube challenge) at 10 minutes had similar symptoms and response to antihistamines as those patients with positive ice-cube-challenge test. In addition, our group of patients with cold urticaria had a strikingly high rate of asthma (46.7%) and allergic rhinitis (50%). The rate of family history of atopic diseases was even higher (89.3%).Conclusions. Cold urticaria occurs in children and may be associated with anaphylaxis. In our series, no secondary causes were found. All patients with cold urticaria and their parents should be cautioned regarding the risk of anaphylaxis and provided with an epinephrine autoinjector. Pediatrics 2004;113:e313-e317. URL: http://www.pediatrics.org/cgi/content/full/113/4/e313; cold urticaria, anaphylaxis, cold-stimulation test.ABBREVIATION. H1, histamine 1 receptor. C old urticaria is characterized by the development of urticaria and/or angioedema after cold exposure. 1 It is an uncommon form of physical urticaria and thought to be rare in children. Of patients with cold urticaria, Ͼ90% have idiopathic (essential) cold urticaria. [2][3][4] The remainder are mostly secondary to cryoglobulinemia. A rare form of the disease, known as delayed cold-induced urticaria, is characterized by the delayed expression of urticaria and angioedema 9 to 18 hours after cold exposure. It is probably inherited as an autosomal dominant condition. 5 Another syndrome known as familial cold urticaria is characterized by the development of intermittent rash (not urticaria), fever, arthralgia, and conjunctivitis 2.5 hours after generalized exposure to cold. It is inherited as an autosomal dominant condition. Patients with this syndrome were identified recently to have mutations in chromosome 1q44. 6 The prevalence and course of cold urticaria are not well defined. The most common method to confirm the diagnosis is the ice-cube-challenge test. It entails the application of an ice cube on the skin for nonstandardized time intervals followed by a period of rewarming. Approximately 20% of patients with cold urticaria have a negative ice-cube-challenge test. 4 A serious and interesting feature of cold urticaria is anaphylaxis. It is observed in one third to one half of adult patients. 3,4,7 Anaphylaxis has resulted in several deaths either ...
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