Clinical Features and Anaphylaxis in Children With Cold Urticaria

Abstract

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 ...

Acquired cold urticaria: Clinical features, particular phenotypes, and disease course in a tertiary care center cohort

“…Although clinical features of cold urticaria seem to be similar regardless of the age at symptom onset, in our series, patients with pediatric-onset ACU showed some differences; ACU was more difficultly induced through the ice cube test or the TempTest, and a complete resolution of symptoms was less likely to be achieved. These results are consistent with a previous pediatric study 3 in which complete symptom resolution was achieved in only 2 of 30 (7.4%) children with cold urticaria, and 41% of the patients had negative CSTs. Similarly, Santaolalla et al 11 noted a full recovery of symptoms in only 1 of 12 pediatric patients analyzed.…”

“…The frequency of atypical ACU in previous series varied between 8% and 49%. 3,9,10,12 Although the knowledge of this entity, especially regarding its natural history, is currently scarce, it has been postulated that these patients would need total body cooling to reflect cold urticaria symptoms. 1 A previous report also suggested a higher incidence of systemic reactions in these patients (10 patients with atypical ACU were analyzed, 6 presented with type II reactions and 2 with type III reactions).…”

“…Rates of satisfactory clinical response with antihistamines ranged from 60% to 70% in previous series. 3,11,16 In contrast to cyclosporine, omalizumab was found to be an effective therapeutic alternative for patients with refractory ACU. 4,17,18 Importantly, the presence of other family members with similar symptoms should always be ruled out in patients with ACU, because specific treatments (eg, antieinterleukin-1) have demonstrated effectiveness in the management of familial cold-induced urticaria syndromes.…”

“…[1][2][3] A diagnosis of ACU is typically made in patients with a clinical history of wheals or angioedema after cold exposure; the diagnosis should then be appropriately verified with cold stimulation tests (CSTs), the ice cube test being the traditional standardized method. 1,2,4 In addition, objective methods, such as the TempTest (Emosystems, Berlin, Germany)da Peltier elemente based provocation device used for the evaluation of the critical temperature threshold (CTT) and the cold stimulation time threshold (CsTT)dare helpful for assessing ACU severity and monitoring therapy efficacy.…”

Cold urticaria and infectious mononucleosis in children

“…Cold urticaria can be classified according to its response to cold challenge test: if the ice cube test turns positive, it is primary or secondary, according to its aetiology; if the response is atypical (late or in a distant place from where stimulus was applied) or negative with suggestive clinical symptoms, it is atypical acquired urticaria 1,3,6 . Primary or idiopathic acquired urticaria is the most common form: Neittaanmäki 7 found a prevalence of 96 % in 220 patients studied with cold urticaria, Santaolalla et al 8 in a review of 12 paediatric cases found an infection cause in only one case and Alangari et al 9 , in a series of 30 children with cold urticaria didn't find any secondary cause.…”