Food allergy, dermatologic diseases, and anaphylaxis
Successful treatment of cold-induced urticaria/anaphylaxis with anti-IgE

https://doi.org/10.1016/j.jaci.2006.04.003Get rights and content

A case of a girl who presented at age 12 years with idiopathic cold urticaria is described. Her reactions to the cold became progressively more severe over a period of approximately 2 years, despite therapy with H1 antagonists and a type 1 receptor for cysteinyl leukotrienes receptor antagonist. She began to experience systemic symptoms on immersion in ocean water. She was atopic and had moderate persistent asthma. A trial of anti-IgE resulted in complete resolution of her urticaria and its associated manifestations. These findings should prompt a reexamination of the potential pathogenetic role played by IgE and its high-affinity receptor on mast cells in idiopathic cold urticaria.

Section snippets

Case presentation

A 12-year-old girl presented for evaluation of a repetitive pruritic rash occurring reproducibly after exposure to cold temperatures. She had been in her usual state of health until several months after moving to the Northeastern United States from Florida. Episodes of rash initially were typified by raised, erythematous, intensely pruritic skin lesions that were confined to cold-exposed areas, especially after contact with cool water or cold surfaces. The lesions developed within minutes,

Differential diagnosis

This otherwise healthy 11-year-old presented with cold-induced cutaneous reactions consistent with urticaria. These episodes were steadily progressive in frequency and severity over a roughly 2-year period. Systemic symptoms (chest tightness) had occurred with cool water immersion, and symptoms of oropharyngeal pruritus and possibly pharyngeal edema had ensued with ingestion of cool liquids. The result of an “ice cube test” was floridly positive. She was atopic and had moderate persistent

Laboratory workup

Complete blood cell revealed a hemoglobin value of 13.5 g/dL, a hematocrit value of 41%, and a total white cell count of 7500/mm3. Differential showed 51% neutrophils, 37% lymphocytes, 8% monocytes, and 4% eosinophils (total eosinophil count of 300/mm3). The erythocyte sedimentation rate was 4. The total IgE level was 1078 IU/mL. Cryoglobulins, cryofibrinogen, and cold agglutinins were not detectable.

Tentative diagnosis with discussion of pathogenesis

This patient's clinical presentation was consistent with the diagnosis of idiopathic cold urticaria.1 Cold urticaria accounts for approximately 3% of all cases of chronic urticaria.2 Both young adults and children are affected,2, 3 and cases have been documented in infants as young as 6 months.3 In a series of 30 pediatric patients, the median age of onset was 7 years (range, 0.5-14.5 years).3 The course tends to be chronic, with most patients experiencing symptoms for years, regardless of the

Diagnosis and discussion of management plan

The clinical history of the reactions, the absence of systemic illness and cryoproteins, and the positive ice cube challenge test response (application of an ice cube to the forearm for 5 minutes, followed by 10 minutes of observation) confirmed the diagnosis of idiopathic cold urticaria in this patient (type 3 according to the classification of Wanderer et al1). Despite increasing doses of cetirizine (to 10 mg twice daily), concomitant use of montelukast, and trials of other antihistamines,

Summary

Although cold urticaria is associated with cryoproteins and associated underlying systemic disease in a small patient subgroup, most patients have an idiopathic variant. This is especially true for children. The diagnostic workup should be tailored based on patient age and associated symptoms and signs. Treatment with H1 antagonists is helpful to most, but not all, patients and reflects the involvement of other mediators, including cysteinyl leukotrienes. The risk of mortality with water

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    Supported by National Institutes of Health grants AI-48802, AI-52353, AI-31599, and HL-36110 and by grants from the Charles Dana Foundation and the Vinik Family Fund for Research in Allergic Diseases.

    Disclosure of potential conflict of interest: J. A. Boyce has consultant arrangements with Altana and Tanox and is on the speakers' bureau for Genentech/Novartis, Merck, and GlaxoSmithKline.

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