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Familial Cold Auto-inflammatory Syndrome FCAS / FCU

FCAS / FCU, Familial Cold Auto-inflammatory Syndrome, is a condition that causes episodes of fever, skin rash, and joint pain after exposure to cold temperatures. These episodes usually begin in infancy and occur throughout life.

Symptoms occur after cold exposure of an hour or more, although for some only a few minutes of exposure is required. Symptoms may be delayed for up to a few hours after the cold exposure. Episodes last an average of 12 hours, but may continue for up to 3 days.

The most common symptom that occurs during an episode is an itchy or burning rash. The rash usually begins on the face or extremities and spreads to the rest of the body. Occasionally swelling in the extremities may occur.  In addition, episodes are characterized by fever, chills, and joint pain, most often affecting the hands, knees, and ankles. Redness in the whites of the eye (conjunctivitis), sweating, drowsiness, headache, thirst, and nausea may also occur during an episode of this disorder.

Links for further research:
Amyloidosis 
Dermnetnz
National Institute for Health
Genetic Summary
More Genetic Information

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Nuggets of Wisdom

Yes, there is such a thing as having an allergic type reaction to temperatures. This is defined as a physical urticaria. Other physical urticarias include Solar, Aquagenic, Pressure, Vibration and Exercise.

Most reactions are pseudo-allergic. By definition, an allergy involves inhaling or consuming an allergen. Physical urticarias have no known allergen. Despite the terminology and medical definitions, systemic reactions can be life threatening.

Cold has an arbitrary definition based on an individual feeling. For a person with a cold urticaria, cold can be defined as any temperature cooler than their own body temperature.

You do not have to be cold to have a reaction to the cold; contact with cold can trigger a reaction.

You can have an allergic type reaction to both cold and heat simultaneously.

Most reactions considered anaphylactic are really anaphylactoid by definition.

Moving to a warmer climate as a treatment for Cold Urticaria is a myth. Warmer climates present their own issues for those with Cold Urticaria.

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