Food allergy, dermatologic diseases, and anaphylaxisSuccessful treatment of cold-induced urticaria/anaphylaxis with anti-IgE
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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Cited by (185)
Cold Urticaria Syndromes: Diagnosis and Management
2023, Journal of Allergy and Clinical Immunology: In PracticeTaiwanese dermatological association consensus for the definition, classification, diagnosis, and management of urticaria: A 2021 update
2022, Journal of the Formosan Medical AssociationCitation Excerpt :Due to the differences in clinical responses and economic considerations, the personalized dosing intervals and continued treatment during extension periods should be tailored to suit the patients’ needs.24–26 Supplementary Table 1 provides a list of alternative anti-inflammatory agents that can be considered for use in certain cases of CU (approved by 100% of the consensus meeting attendees who joined the voting process), including information from the EAACI urticaria guideline regarding the degree of research evidence for each agent and the strength of the corresponding EAACI recommendation; the British Society for Allergy and Clinical Immunology grades and indications for the agents are likewise included.27–37 Acute urticaria differs from CU by spanning no more than 6 weeks.
Prevalence, Management, and Anaphylaxis Risk of Cold Urticaria: A Systematic Review and Meta-Analysis
2022, Journal of Allergy and Clinical Immunology: In PracticeOmalizumab in cold urticaria in children: Retrospective case series of 13 patients, review of the literature
2021, Annales de Dermatologie et de VenereologieOmalizumab in chronic inducible urticaria: A retrospective, real-life study
2021, Annales de Dermatologie et de VenereologieA single-center experience of treating 19 patients with chronic cold urticaria
2021, Journal of Allergy and Clinical Immunology: In Practice
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.