"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.

Some of these include NOMIDS and MWS"

(from http://pweb.netcom.com/~islnddrm/familial.htm accessed 12.30.06)

Read a very user-friendly site for FCU here

See Hoffman (2003/2005) defining article here.

"Hereditary episodic fever syndromes:
A special opening session was dedicated to inherited childhood episodic fever syndromes, discussing both clinical and basic science aspects. Dan Kastner (USA) discussed the molecular basis for these syndromes, discovered only during the last decade. In most of these syndromes the genetic defect results in a pro-inflammatory state. Dr. Kastner dubbed these syndromes as auto-inflammatory. Two of the diseases, Familial Mediterranean Fever (FMF) and hyperimmunoglobulin IgD (HIDS) syndrome have a autosomal recessive mode of transmission. The other three syndromes, Muckle-Wells syndrome (MWS), tumor necrosis factor associated periodic syndrome (TRAPS) and familial cold urticaria (FCU) have an dominant mode of transmission. The causes of periodicity and the environmental stimuli of attacks are unclear, besides cold that triggers episodes of FCU."

(from http://www.pedrheumonlinejournal.org/issues1/PRESmeeting.html accessed 12.30.2006)