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$Pretitle{}
$Title{Urticaria Pigmentosa}
$Subject{Urticaria Pigmentosa Xanthelasmoidea Infantile Mastocytosis Perstans
Hemorrhagica Urticaria Papular Urticaria Pigmentosa Nodular Urticaria
Pigmentosa}
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Copyright (C) 1986 National Organization for Rare Disorders, Inc.

192:
Urticaria Pigmentosa

** IMPORTANT **
It is possible the main title of the article (Urticaria Pigmentosa) is
not the name you expected.  Please check the SYNONYMS listing to find the
alternate names and disorder subdivisions covered by this article.

Synonyms

     Xanthelasmoidea
     Infantile Mastocytosis
     Perstans Hemorrhagica Urticaria

DISORDER SUBDIVISIONS

     Papular Urticaria Pigmentosa
     Nodular Urticaria Pigmentosa

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.


The most common form of Urticaria Pigmentosa is the papular form which is
characterized by itchiness, brown spots (macules), and smooth, slightly
elevated areas of different color appearing on the skin.  There is
progression to pigmentation, followed by the formation of erythema (redness),
and edema when the lesions are rubbed.  In the nodular form of Urticaria
Pigmentosa, scarring is especially prominent.

Symptoms

Characteristically, persons with Urticaria Pigmentosa have skin lesions which
may be itchy due to an excess of mast cells in the skin.  Malaise, anorexia
(loss of appetite), abdominal pain, flatulence, diarrhea, and pain in the
back, chest and joints also may be present.

Causes

The cause of Urticaria Pigmentosa is unknown.  The disorder may be due to an
allergic, pseudoleukemic, or metabolic disturbance.  Most cells release
histamine which causes many of the symptoms.

Affected Population

Onset of Urticaria Pigmentosa is generally in the first year of life.
Lesions usually disappear by adolescence except when Systemic Mastocytosis is
present (see section on related disorders).

Related Disorders

In Systemic Mastocytosis there are multiple confluent spots, papules and
nodules in the skin, and pigmentation may be less.  Extensive involvement of
the mucous membranes of the mouth, nose and rectum, and enlargement of the
spleen, liver and lymph nodes also may be present.  Edema and shock-like
episodes may also occur.

Therapies:  Standard

Treatment of Urticaria Pigmentosa is symptomatic and supportive.

Therapies:  Investigational

Urticaria Pigmentosa has been treated experimentally with oral disodium
cromoglycate, the H-2 antihistamine cimetidine (sometimes combined with the
H-1 antihistamine chlorpheniramine or the anticholinergic drug propantheline)
and with ketotifen.

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 Urticaria Pigmentosa, please contact:

     National Organization for Rare Disorders (NORD)
     P.O. Box 8923
     New Fairfield, CT  06812-1783
     (203) 746-6518

     The National Arthritis and Musculoskeletal and Skin Diseases Information
Clearinghouse
     Box AMS
     Bethesda, MD  20892
     (301) 495-4484

References

THE MERCK MANUAL 15th ed:  R. Berkow, et al:  eds; Merck, Sharp & Dohme
Research Laboratories, 1987.  P. 310.

CECIL TEXTBOOK OF MEDICINE, 18th ed.:  James B. Wyngaarden, and Lloyd H.
Smith, Jr., Eds.:  W. B. Saunders Co., 1988.  Pp. 1948-51, 2334-5.