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Cold urticaria (essentially meaning cold hives) is a disorder in which large red welts called hives (urticaria) form on the skin after exposure to a cold stimulus. [1] The hives are usually itchy and often the hands, feet and other parts of the body will become itchy and swollen as well.
Urticaria is a vascular reaction of the skin characterized by the appearance of wheals, which are firm, elevated swellings of the skin. [105] Angioedema , which can occur alone or with urticaria, is characterized by a well-defined, edematous swelling that involves subcutaneous tissues, abdominal organs, or upper airway.
The first outbreak of urticaria can lead to other reactions on body parts not directly stimulated, scraped, or scratched. In a normal case, the swelling will decrease without treatment within 15–30 minutes, but, in extreme cases, itchy red welts may last anywhere from a few hours to days.
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The common form of cold urticaria demonstrates itself with the rapid onset of hives on the face, neck, or hands after exposure to cold. Cold urticaria is common and lasts for an average of five to six years. The population most affected is young adults, between 18 and 25 years old.
It encompasses a spectrum of three clinically overlapping autoinflammatory syndromes including familial cold autoinflammatory syndrome (FCAS, formerly termed familial cold-induced urticaria), the Muckle–Wells syndrome (MWS), and neonatal-onset multisystem inflammatory disease (NOMID, also called chronic infantile neurologic cutaneous and ...
This condition is characterised by cold induced urticaria, autoimmunity, atopy and humoral immune deficiency. [2] The humeral immune deficiency results in recurrent bronchopulmonary infections. Cutaneous granulomas may also occur. [citation needed] The urticaria usually appears within 12 months of birth but may appear immediately after birth. [3]
The disease is caused in 60% of cases by a mutated gene called CIAS1 that is known to be involved in other syndromes that appear somewhat similar, such as Muckle–Wells syndrome and familial cold urticaria. In many patients, the parents do not have the same mutation, indicating the problem was not inherited, even though it is a genetic disease.