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$Unique_ID{BRK04302}
$Pretitle{}
$Title{Urticaria, Cold}
$Subject{Urticaria, Cold Idiopathic Cold Urticaria (Familial or Acquired)
Primary Idiopathic Cold Urticaria Raynaud's Disease Cold Agglutinin Disease
Paroxysmal Cold Hemoglobinuria }
$Subject{}
$Volume{}
$Log{}
Copyright (C) 1988, 1989 National Organization for Rare Disorders, Inc.
565:
Urticaria, Cold
** IMPORTANT **
It is possible that the main title of the article (Cold Urticaria) is not
the name you expected. Please check the SYNONYM listing to find the
alternate names and disorder subdivisions covered by this article.
Synonyms
DISORDER SUBDIVISIONS
Idiopathic Cold Urticaria (Familial or Acquired)
Primary Idiopathic Cold Urticaria
Information on the following diseases can be found in the Related
Disorders section of this report:
Raynaud's Disease
Cold Agglutinin Disease
Paroxysmal Cold Hemoglobinuria
General Discussion
** REMINDER **
The information contained in the Rare Disease Database is provided for
educational purposes only. It should not be used for diagnostic or treatment
purposes. If you wish to obtain more information about this disorder, please
contact your personal physician and/or the agencies listed in the "Resources"
section of this report.
Cold Urticaria is a chronic, reactive skin disorder. It is probably the
most common form of physical urticaria. Major symptoms may include abnormal
reddening of the skin (erythema), hives and itching after exposure of the
skin to cold temperatures.
Symptoms
In Cold Urticaria the skin has an abnormal reaction to cold. The skin
usually turns red, develops welts and itching. This may be accompanied by
fever, headache, anxiety, tiredness, and sometimes even fainting. Some
persons may also have palpitations or wheezing.
Idiopathic Cold Urticaria may be familial or acquired. Familial Cold
Urticaria is a rare condition inherited as an autosomal dominant trait. It
consists of burning red skin patches which develop approximately thirty
minutes after being exposed to cold weather. This condition may persist for
up to forty-eight hours after exposure. It may be accompanied by fever,
headache, tiredness, pain in the joints (arthralgia) and the presence of
excessive white blood cells (leucocytosis) in the blood.
Acquired Cold Urticaria consists of Primary Acquired Cold Urticaria,
Delayed Cold Urticaria, Localized Cold Urticaria, Reflex Cold Urticaria or
Secondary Cold Urticaria, which are explained below:
Primary Acquired Cold Urticaria can occur five to thirty minutes after
exposure to cold. The reaction may occur in the cold itself, but more often
during the rewarming phase. Itching and reddening of the skin may develop
first, followed by a burning sensation. Hives appear, usually lasting thirty
minutes. The affected person may also experience headache, palpitations,
wheezing or fainting.
Delayed Cold Urticaria may appear several hours after contact with the
cold.
Localized Cold Urticaria has been reported to occur after exposure to
cold at the sites of previous ragweed injections for allergies or ladybug
bites.
Reflex Cold Urticaria is characterized by widespread appearance of welts
occuring in response to a drop in body temperature after localized exposure
to cold applications (e.g. an ice pack).
Secondary Cold Urticaria can occur in connection with various blood
disorders associated with viral infections such as Mononucleosis or Syphilis.
Causes
Cold Urticaria can occur for unknown (idiopathic) reasons, or it may be
transmitted as an autosomal dominant trait. (Human traits including the
classic genetic diseases, are the product of the interaction of two genes for
that condition, one received from the father and one from the mother. In
dominant disorders a single copy of the disease gene (received from either
the mother or father) will be expressed "dominating" the other normal gene
and resulting in appearance of the disease. The risk of transmitting the
disorder from affected parent to offspring is fifty percent for each
pregnancy regardless of the sex of the resulting child.)
It may also be a disease of the autoimmune system. (Autoimmune disorders
are caused when the body's natural defenses (antibodies) against invading
organisms suddenly begin to attack perfectly healthy tissue.)
Exposure of the skin to cold triggers symptoms of the disorder such as
cold weather, swimming in cold water or even a cold bath.
Affected Population
Cold Urticaria affects males and females in equal numbers.
Related Disorders
Symptoms of the following disorders can be similar to those of Cold
Urticaria. Comparisons may be useful for a differential diagnosis:
Raynaud's Disease is a vascular disorder that is triggered by exposure to
cold. It is characterized by spasms of blood vessels occurring especially in
the fingers and toes. Intermittent attacks of pain, pallor or blue coloring
(cyanosis) of the fingers or toes are precipitated by exposure to cold or by
emotional upsets. The attacks last for minutes to hours, but are rarely
severe enough to result in tissue loss. Rewarming the affected digits
results in normal blood circulation and a return to normal color and
sensation. Onset usually occurs in the first or second decade of life. (For
more information on this disorder, choose "Raynaud" as your search term in
the Rare Disease Database).
Cold Agglutination Disease is a blood disorder which occurs when the
temperature of the blood is below body temperature. It is most pronounced
below 25 C. Although it is seen occasionally in the blood of apparently
healthy persons, it is more frequent in individuals with scarlet fever,
staphylococcal infections, primary atypical pneumonia, certain hemolytic
anemias, and trypanosomiasis.
Paroxysmal Cold Hemoglobinuria is a disorder that makes the red blood
corpuscles abnormally susceptible to antibodies which try to destroy them.
It is triggered by exposure to cold. (For more information on thia disorder,
chooose "Hemoglobinuria" as your search term in the Rare Disease Database.)
Therapies: Standard
Treatment of Cold Urticaria may include the use of the drugs epinephrine,
diphenhydramine, cyproheptadine, hydrochloride and cetirizine. Symptoms may
be prevented with the use of warm clothing during cold weather. The
avoidance of cold baths, swimming in cold water, etc., is recommended since
loss of consciousness may occur, possibly resulting in drowning.
Therapies: Investigational
This disease entry is based upon medical information available through
November 1988. Since NORD's resources are limited, it is not possible to
keep every entry in the Rare Disease Database completely current and
accurate. Please check with the agencies listed in the Resources section for
the most current information about this disorder.
Resources
For more information on Cold Urticaria, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
NIH/National Institute of Allergy & Infectious Diseases (NIAID)
9000 Rockville Pike
Bethesda, MD 20892
(301) 496-5717
Asthma & Allergy Foundation of America
1717 Massachusetts Avenue NW, Suite 305
Washington, DC 20036
(202) 265-0265
For information about Raynaud's Disease only:
Raynaud's Association Trust
c/o Bladon Crescent
Alsager, Cheshire 5T7 2BG
England
For genetic information and genetic counseling referrals:
March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
(914) 428-7100
Alliance of Genetic Support Groups
35 Wisconsin Circle, Suite 440
Chevy Chase, MD 20815
(800) 336-GENE
(301) 652-5553
References
INTERNAL MEDICINE, 2nd Ed.: Jay H. Stein, ed.-in-chief; Little, Brown and
Co., 1987. Pp. 945, 1058.
CLINICAL CHARACTERISTICS OF COLD-INDUCED SYSTEMIC REACTIONS IN THIS
COMPLICATION AND A PROPOSAL FOR A DIAGNOSTIC CLASSIFICATION OF COLD
URTICARIA. A.A. Wanderer, et al.; J Allergy Clin Immunol (September, 1986,
issue 78 (3 Pt. 1)). Pp. 417-423.
COLD URTICARIA: RELEASE INTO THE CIRCULATION OF HISTAMINE AND EOSINOPHIL
CHEMOTACTIC FACTOR OF ANAPHYLAXIS DURING COLD CHALLENGE. N.A. Soter, et al.;
N Engl J Med (March, 1975, issue 294 (13)). Pp. 687-690.
INHIBITING EFFECT OF CETIRIZINE ON HISTAMINE-INDUCED AND 48/80-INDUCED
WHEALS AND FLARES, EXPERIMENTAL DERMOGRAPHISM, AND COLD-INDUCED URTICARIA.
L. Juhlin et al.; J Allergy Clin Immunol (October, 1987, issue 80, (4)). Pp.
599-602.