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$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.