Case Reports

Diagnosis and Management of Cold Urticaria

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Cold urticaria is a physical urticaria characterized by a localized or systemic eruption of papules upon exposure of the skin to cold air, liquids, and/or objects. In some cases, angioedema and anaphylaxis also may occur. The symptoms of cold urticaria can have a negative impact on patients’ quality of life. Second-generation H1 antihistamines are the first line of treatment in cold urticaria; however, patients who are unresponsive to initial treatment with H1 antihistamines may require further management options. Avoidance of cold exposure is the most effective prophylactic measure. In mild to moderate cases, the primary goal of therapy is to improve the patient’s quality of life. In more severe cases, treatment measures to protect the patient’s airway, breathing, and circulation may be necessary. We report the case of a 23-year-old man with cold urticaria who was refractory to initial therapy with H1 antihistamines. A review of the literature also is provided.

Practice Points

  • Cold urticaria is a physical urticaria characterized by a localized or systemic eruption of papules upon exposure of the skin to cold air, liquids, and/or objects.
  • Symptoms of cold urticaria, which range from erythema, pruritus, and hives to angioedema and sometimes anaphylaxis, may be debilitating for patients; therefore, effective treatment is required to improve quality of life.
  • First-line treatment for cold urticaria includes second-generation H1 antihistamines at up to 4 times the standard dosage.


 

References

Cold urticaria is a rare condition characterized by a localized or systemic eruption of papules upon exposure of the skin to cold air, liquids, and/or objects. In some cases, angioedema and anaphylaxis can occur. The wheal-and-flare reaction results from a localized or systemic release of histamine, leukotrienes, and various other proinflammatory mast cell mediators. Cold urticaria can be acquired or follow an autosomal-dominant familial transmission pattern. Acquired cold urticaria often presents in young adulthood with a mean duration of 4 to 5 years and remission or improvement of symptoms after 5 years in 50% of cases.1 The familial variant most commonly presents in early childhood and endures throughout the patient’s life.2 Cold urticaria generally is classified as acute or chronic if symptoms persist for more than 6 weeks. Pharmacologic therapies with prophylactic effects that may reduce the intensity of symptoms or inhibit their development include antihistamines, leuko-triene receptor antagonists, biologics, and glucocorticoids. We present the case of a 23-year-old man with cold urticaria that was refractory to initial treatment with H1 antihistamines along with a review of the literature.

Case Report

A 23-year-old man presented to the dermatology clinic for evaluation of recurrent burning, itching, and sometimes development of a painful rash on the face, neck, and arms of 2 years’ duration that typically occurred following exposure to cold, wind, and rain. He also developed symptoms in warm weather when exposed to wind while sweating. His medical history was remarkable for asthma, which was not active. He was not taking any medications and had no known drug or environmental allergies. No other members of his household developed similar symptoms. His only successful means of prevention was to stay indoors, which thereby limited his activities.

Physical examination of the dorsal hands following an ice cube test revealed numerous 3- to 5-mm urticarial papules with surrounding erythema (Figure).

Urticarial papules on the right hand following an ice cube test.

Urticarial papules on the right hand following an ice cube test.

Following the initial evaluation, the patient was treated unsuccessfully with a mix of first- and second-generation antihistamines in gradually increasing doses to a maximum dose of loratadine 20 mg once daily, cetirizine 20 mg once daily, and hydroxyzine 20 mg once daily. A course of montelukast 10 mg once daily was started in addition to the antihistamines and led to a reduction in the severity of the lesions but not the frequency and did not relieve the burning sensation; the patient subsequently discontinued therapy. Next, a trial of cyclosporine was attempted, but the patient reported that it caused emesis and subsequently discontinued treatment. The patient also did not tolerate prednisone. He eventually decided to treat his symptoms with lifestyle choices only, such as making sure to be well covered in cold temperatures.

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