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Familial cold urticaria (cold-related hives)


Description

A rare inherited disorder that causes hives (urticaria) after exposure to cold. Other symptoms such as fever and joint pain may be prominent. These episodes are triggered by exposure to cold and may last hours to a few days. Familial cold urticaria is also known as familial cold auto-inflammatory syndrome. The first episodes usually occur during infancy and last throughout a person's life.

Symptoms

Rash (hives), fever, itching, and joint pain after exposure to cold.

Tests

Common tests used for diagnosis and treatment

Workup:
A history and physical exam will be performed. Genetic testing may be recommended.

Other Specific Tests: Allergy testing

Specialists:
Internal Medicine, Pediatrics, Family Practice, Allergy and Immunology, Pediatric Allergy and Immunology

Treatment

Treatment includes steroids, and antihistamines (such as diphenhydramine/Benadryl) for the itching. When symptoms are significant and not well controlled with antihistamines alone, injectable medications such as anakinra (Kineret) or canakinumab (Ilaris) may be effective.
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