What is Familial Cold Urticaria?
Familial Cold Urticaria (FCU) is an immunologic condition in which the affected person develops several symptoms when they are exposed to cold environments. Approximately 1-4 hours after a cold exposure rash, fever, chills, joint pains, and red eyes develop. Other less common symptoms include extreme thirst, drowsiness, nausea, and headaches. An “attack” often lasts 24-48 hours. The rash is not itchy, but is often described as painful or “burning.” FCU greatly affects the daily lives of the people who have it, but the “attacks” are not life threatening. Most people with FCU live very long lives without any other medical problems. However, there are some families with FCU who develop kidney disease late in life that can be severe. Another name for FCU is FCAS (Familial Cold Auto-inflammatory Syndrome). FCAS is caused by a gene mutation in the CIAS gene. There is a continuum of syndromes with a CIAS gene mutation that are related to FCAS, but have different symptoms.
