Cold Urticaria is an allergy where hives or large red welts form on the skin
after exposure to a cold stimulus. The welts are usually itchy and often the
hands and feet will become itchy and swollen as well. Hives vary in size from
about 7mm in diameter to as big as about 27mm diameter or larger. The disease is
classified as chronic when hives appear for longer than 6 weeks; they can last
for life, though their course is often unpredictable. This disorder, or perhaps
two disorders with the same clinical manifestations, can be inherited or
acquired. The acquired form is most likely to occur between ages 18–25,
although it can occur as early as 5 years old in some cases.
Types Cold urticaria may be divided into the
following types: = Primary cold contact urticaria=
Primary cold contact urticaria is a cutaneous condition characterized by
weals, and occurs in rainy, windy weather, and after swimming in cold
water and after contact with cold objects, including ice cubes.
= Secondary cold contact urticaria= Secondary cold contact urticaria is a
cutaneous condition characterized by weals, due to serum abnormalities such
as cryoglobulinemia or cryofibrinogenemia are extremely rare,
and are then associated with other manifestations such as Raynaud's
phenomenon or purpura. = Reflex cold urticaria=
Reflex cold urticaria is a cutaneous condition in which generalized cooling
of the body induces widespread wealing. = Familial cold urticaria=
Familial cold urticaria is an autosomal dominant condition characterized by
rash, conjunctivitis, fever/chills and arthralgias elicited by exposure to cold
- sometimes temperatures below 22 °C. It has been mapped to CIAS1 and is a
slightly milder member of the disease family including Muckle–Wells syndrome
and NOMID. It is rare and is estimated as having a prevalence of 1 per million
people and mainly affects Americans and Europeans.
FCAS is one of the cryopyrin-associated periodic syndromes caused by mutations
in the CIAS1/NALP3 gene at location 1q44. The disease was described in The
Lancet Volume 364 by Hoffman H.M. et al. The effect of FCAS on the quality of
life of patients is far reaching. A survey of patients in the United States
in 2008 found, "To cope with their underlying disease and to try to avoid
symptomatic, painful, flares patients reported limiting their work, school,
family, and social activities. Seventy-eight percent of survey
participants described an impact of the disease on their work, including
absenteeism and impaired job advancement; frequently, they quit their
job as a consequence of their disease". Treatment using anakinra has been shown
effective for FCAS, although this does mean daily injections of the
immunosuppressant into an area such as the lower abdomen. The monoclonal
antibody canakinumab is also used. Symptoms
When the body is exposed to the cold in individuals afflicted by the condition,
hives appear and the skin in the affected area typically becomes itchy.
Hives result from dilation of capillaries which allow fluid to flow
out into the surrounding tissue which is the epidermis.They resolve when the body
absorbs this fluid. The border of a hive is described as polycyclic, or made up
of many circles, and changes as fluid leaks out and then is absorbed. Pressing
on a hive causes the skin to blanch distinguishing it from a bruise or
papule. Hives can last for a few minutes or a few days, and vary from person to
person. Also a burning sensation occurs. During a severe reaction, hypotension,
which can be life-threatening, can occur. A serious reaction is most likely
to occur if the hives occur with less than 3 minutes of exposure.
Cause The hives are a histamine reaction in
response to cold stimuli, including a drastic drop in temperature, cold air,
and cold water. There are many causes for cold hives, most are idiopathic.
Some rare conditions can cause cold hives, and it can be useful to test for
these conditions if the cold hives are in any way unusual.
Scientists from the USA National Institutes of Health have identified a
genetic mutation in three unrelated families that causes a rare immune
disorder characterized by excessive and impaired immune function: immune
deficiency, autoimmunity, inflammatory skin disorders and cold-induced hives.
"The mutation discovered occurs in a gene for phospholipase C-gamma2, an
enzyme involved in the activation of immune cells. The investigators have
named the condition PLCG2-associated antibody deficiency and immune
dysregulation, or PLAID." Diagnosis
Diagnosis is typically obtained by an allergist or other licensed practitioner
performing a cold test. During the cold test, a piece of ice is held against the
forearm, typically for 2–3 minutes. A positive result is a specific looking
mark of raised red hives. The hives may be the shape of the ice, or it may
radiate from the contact area of the ice.
Treatment The most important treatment is to stay
warm. Some air conditioned rooms and pools may be too cold for the skin.
Warming up immediately after exposure to cold temperatures usually helps control
the hives before they get worse. Although the hives do not
instantaneously disappear after warming the affected area(s), warming up
afterwards reduces the time it takes for the hives to go away. Some patients
report that spreading butter or vegetable shortening on their affected
areas reduces the risk of future eruptions.
Allergy medications containing antihistamines such as Benadryl, Zyrtec,
Claritin, Periactin and Allegra may be taken orally to prevent and relieve some
of the hives. For those who have severe anaphylactic reactions, a prescribed
medicine such as doxepin, which is taken daily, should help to prevent and/or
lessen the likelihood of a reaction and thus, anaphylaxis. There are also
topical antihistamine creams which are used to help relieve hives in other
conditions, but there is not any documentation stating it will relieve
hives induced by cold temperature. Cold hives can result in a potentially
serious, or even fatal, systemic reaction. People with cold hives may
have to carry an injectable form of epinephrine for use in the event of a
serious reaction. The best treatment for this allergy is
avoiding exposure to cold temperature. Ebastine has been proposed as an
approach to prevent acquired cold urticaria.
Situations when reactions may occur Cold weather: Exposure to cold or cool
air for more than a few minutes can trigger a reaction. Avoid long walks
outdoors during cold weather. Be prepared to bundle up and cover exposed
skin as long as the weather remains cool. Experiment to find the right
number of layers. Sweat: A reaction may even occur on a
warm day when there is sweat on the skin. If there is a breeze it will
rapidly cool the skin and create hives. Getting warm quickly by drying the skin
and covering up will help to control the hives before they get too bad. Using or
carrying a towel while exercising and wiping off sweat before it has a chance
to dry is an effective means of preventing hives from occurring.
Cold/Cool IV Lines: While in the hospital, inserting IV lines that have
been refrigerated or are cool into individuals who are allergic to cold
temperature can/may result in pain within the limb the IV is placed and a
line of hives may appear within a radius of 6-8 inches from the IV site in the
pattern of a line going up the limb. Notify your doctor/nurse of your
condition so they will be able to use a machine which warms IV lines before they
enter your body Swimming: Swimming can be especially
dangerous as the rapid heart rate combined with the onset of hives can
lead to hypotension. Taking an allergy medication before entering the water and
conserving energy until your body adjusts to the temperature may help to
prevent the hypotension. Take an antihistamine if you choose to go
swimming; preferably a non-drowsy antihistamine, and have an individual
with you at all times Air Conditioning: Entering a cool
building during a summer day can result in an allergic reaction to exposed areas
of skin, congestion from breathing the cool air and possibly feeling fatigued.
Carrying jackets with you on warm days is a good idea in case you enter an air
conditioned building. Cold Foods/Drinks: Eating or drinking
cold or cool substances such as ice cream or even iced tea may result in the
individuals tongue and surrounding tissues swelling. If eating cold foods
or having cold drinks results in swelling of tissues in and around the
mouth/throat it is advisable to take an antihistamine before consuming them
and/or refraining from eating/drinking them.
Cool/Cold Surfaces: Sitting on sidewalks which are cool, leaning on or grabbing a
cold pole will result in hives forming on the area which had contact with the
cool surfaces. The only treatment for these kinds of situations is trying to
avoid cold surfaces, if you can not avoid them using gloves to touch them or
using a pad to sit on cool sidewalks is helpful
Restriction of Blood Flow: Typing or other activities which tense muscles and
reduce blood flow can cool the body parts enough to cause itching and hives.
Blood Flow: While putting hands or feet in hot water nails turn purple/white
until the body gets used to the temperature.
See also Amyloidosis
Cholinergic urticaria, a similar hives reaction in response to heat
Diascopy Erythema
Goose bumps, an unrelated, normal response to cold temperatures
List of cutaneous conditions Skin lesion
References External links
ColdUrticaria.net Most Informative Site, named a Healthline Top 10 Blog
Cold Urticaria Foundation "Raising Awareness & Support For Cold Urticaria"