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Higher incidence found in regions with colder climate
Prevalent in all races and genders; most commonly seen between ages 10–40 y
Can develop urticaria and/or angioedema with skin cooling and rewarming
Systemic shock-like reactions can occur with whole-body cold exposure (e.g., swimming)
Exposing patients to cold stimulus (e.g., cold room, cold IV or irrigation fluids, cold instruments or devices against the skin)
A subdivision of chronic inducible urticaria (when symptoms last >6 wk)
Accounts for 3–5% of all physical urticarias (urticaria caused by physical stimuli)
Characterized by appearance of urticaria and/or angioedema after cold exposure
Urticaria, which presents as pruritic, superficial erythematous papules or plaques that are blanchable, and angioedema, involving swelling of the deeper dermis, which usually affects face/lips/extremities and tends to be painful
Disease course usually lasting from 5–9 y but may resolve after several months
Symptoms occurring within min after exposure to cold stimulus (cold air/fluids)
Disease: acquired (most common) or familial (rare hereditary disorder)
Acquired: Primary or secondary to an underlying disease process, such as malignancy, cryoglobulinemia, or infection (e.g., HIV, infectious mononucleosis).
After treatment of underlying disease (e.g., treatment with antibiotics): secondary cold urticaria may resolve
Dx: Made with cold stimulation test (ice cube to volar surface of forearm)
If + stimulation test, threshold testing to determine severity of disease
Threshold testing: performed with a computer-aided thermoelectric Peltier device
Primary cold urticaria appears related to skin mast cells sensitization to cold by a serum factor, and is very likely autoantibodies mediated (functional anti-IgE antibodies have been described in pts with ACU).
Sensitized skin mast cells release histamine and other proinflammatory mediators upon interaction with cold stimulus.
Cryoglobulins cause activation in secondary cold urticaria.
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