$Unique_ID{BRK04303} $Pretitle{} $Title{Urticaria, Papular} $Subject{Urticaria, Papular Urticaria Giant Urticaria Hives Lichen Urticatus Angioneurotic Edema Angioedema Quincke Syndrome } $Volume{} $Log{} Copyright (C) 1986, 1987, 1989 National Organization for Rare Disorders, Inc. 248: Urticaria, Papular ** IMPORTANT ** It is possible the main title of the article (Papular Urticaria) is not the name you expected. Please check the SYNONYMS listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Urticaria Giant Urticaria Hives Lichen Urticatus Angioneurotic Edema Angioedema Quincke Syndrome 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. Papular Urticaria, more commonly known as "hives", is characterized by local elevated ridges (wheals) and redness (erythema) of the skin. This condition is usually triggered by allergic reactions to insect bites, sensitivity to drugs, or other environmental causes. Symptoms The first symptom of Papular Urticaria is usually itching (pruritus). This is followed shortly by the appearance of elevated ridges (wheals) that may remain small or become large. The larger wheals tend to be clear in the center, and may be noticed first as large rings of erythema and edema. Ordinarily, crops of hives come and go. A lesion may remain for several hours, then disappear only to reappear elsewhere. Angioedema is a more diffuse swelling of loose tissue under the skin usually affecting the back of hands or feet, lips, genitalia and mucous membranes. Swelling (edema) of the upper airway may produce respiratory distress, and the high-pitched tone of difficult breathing may be mistaken for asthma. Causes Acute Papular Urticaria and Angioedema are essentially exaggerated allergic reactions limited to the skin and tissues right under the skin (subcutaneous tissues). They may be caused by a drug allergy, insect stings or bites, desensitization injections (allergy shots) or ingestion of certain foods (particularly eggs, shellfish, nuts or fruits) by people who are allergic to these substances. In some cases (such as reactions to strawberries) the reaction may occur only after overindulgence, and possibly result from direct toxic histamine release into the blood. Papular Urticaria may accompany, or even be the first symptom of various virus infections including hepatitis, infectious mononucleosis, or German measles (rubella). Some acute reactions are unexplained, even when recurrent. If acute Angioedema is recurrent, progressive, and never associated with Urticaria, a hereditary enzyme deficiency should be suspected. Affected Population Children from 2 to 7 years are most commonly affected by Papular Urticaria, especially in the summertime when the insect population increases. It is more rare in adults, perhaps in part because adults learn to avoid substances and conditions to which they are allergic. Related Disorders Physical Urticaria is a condition in which red allergic skin lesions and itching are produced by exposure to cold temperatures, water or mild trauma. Cholinergic Urticaria is a condition characterized by red spots on the skin, itching and possibly abdominal cramps, diarrhea, faintness, weakness and sweating. It is caused by sensitivity to heat, sunlight, exercise, etc. For more information on these disorders and other types of urticaria, choose "Urticaria" as your search term in the Rare Disease Database.) Therapies: Standard Acute Papular Urticaria is a self-limited condition that generally subsides in 1 to 7 days. Therefore, treatment is chiefly symptomatic. If the cause is not obvious, all nonessential medication should be stopped until the reaction has subsided. Symptoms such as itching and swelling can usually be relieved with an oral antihistamine. Corticosteroids (e.g. prednisone) may be necessary for the more severe reactions, particularly when associated with angioedema. Topical corticosteroids are of no value. Epinephrine should be the first treatment for acute pharyngeal or laryngeal angioedema. This may be supplemented with local (topical) treatment (e.g. nebulized epinephrine) and an intravenous antihistamine. This usually prevents airway obstruction, but making an opening in the trachea (tracheotomy) and oxygen may be necessary. Although the specific cause of chronic Papular Urticaria can seldom be identified and removed, spontaneous remissions usually occur within 2 years in 50% of cases. Control of stressful life situations often helps. Certain drugs (e.g. aspirin) may aggravate symptoms, as can alcoholic beverages, coffee and tobacco. If so, they should be avoided. When Urticaria is produced by aspirin, sensitivity to related compounds, as well as certain food coloring additives, should be investigated. Oral antihistamines are beneficial in most cases. All reasonable measures should be used before resorting to corticosteroids, which are frequently effective, but have significant side effects after chronic use. A few patients with intractable Urticaria are also found to have a hyperthyroid condition. Therapies: Investigational This disease entry is based upon medical information available through March 1987. 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 Papular Urticaria, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 Allergy and Asthma Foundation of America 1835 K Street N.W., Suite P-900 Washington, D.C. 20006 (202) 293-2950 Allergy Information Association 25 Poynter Dr., Suite 7 Weston, Ontario, MR9 1K8 Canada NIH/National Institute of Allergy and Infectious Diseases 9000 Rockville Pike Bethesda, MD 20892 (301) 496-5717 References CECIL TEXTBOOK OF MEDICINE, 18th ed.: James B. Wyngaarden, and Lloyd H. Smith, Jr., Eds.: W.B. Saunders Co., 1988. Pp. 1948-51. THE MERCK MANUAL 15th ed: R. Berkow, et al: eds; Merck, Sharp & Dohme Research Laboratories, 1987. P. 308.