$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.