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    Urticaria pigmentosa

    Mastocytosis; Mastocytoma

    Urticaria pigmentosa is a skin disease that produces patches of darker skin (lesions) andvery baditching. Hives can develop when skin areas are rubbed.

    Causes

    Urticaria pigmentosa occurs in areas where there are too many inflammatory cells (mast cells) in the skin.

    Urticaria pigmentosa is mostcommon in children.It can also occur in adults.

    Symptoms

    The main symptom is brownish patches on skin. Rubbing the skin sore causes a hive-like bump. Younger children may develop ablisterthat is filled with fluid if thebump is scratched.

    The face may also get red quickly (flushed).

    In severe cases, the following symptoms may occur:

    • Diarrhea
    • Fainting (uncommon)
    • Headache
    • Rapid heartbeat (tachycardia)

    Exams and Tests

    • Skin biopsy tolook for a highernumber of mast cells
    • Urine histamine
    • Blood tests for blood cell counts and blood tryptase levels

    Treatment

    Antihistamines medicines can help relieve symptoms such as itching and flushing. Talk to your health care provider about which type of antihistamineto use.

    The health care provider may prescribe other kinds of medicines symptoms fromsevere and unusual forms of urticaria pigmentosa.

    Outlook (Prognosis)

    Urticaria pigmentosa goes away by puberty in about half of the affected children. Symptoms usually get better in others as they grow into adulthood.

    In adults, urticaria pigmentosasystemic mastocytosis. This is a serious condition that can affect the bones, brain and nerves,and digestive system.

    Possible Complications

    The main problems are discomfort from itching, and concern about the appearance of the spots.Other problems such as diarrhea and fainting are rare.

    Certain medicines may trigger flares of urticaria pigmentosa. Discuss these with your doctor.

    Bee stings may also cause a bad allergic reaction in people with urticaria pigmentosa. Ask your doctor if you should carry an injectable epinephrine kit (such as EpiPen or Twinject) touse if you get abee sting.

    When to Contact a Medical Professional

    Call your health care provider if you notice symptoms of urticaria pigmentosa.

    References

    Habif TP. Urticaria and angioedema. In: Habif TP, ed. Clinical Dermatology. 5th ed. St. Louis, Mo: Mosby Elsevier; 2009:chap 6.

    Tharp MD. Mastocytosis. In: Bolognia JL, Jorizzo JL, Schaffer JV, et al, eds.Dermatology. 3rd ed. Philadelphia, Pa: Mosby Elsevier; 2012:chap 118.



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      Tests for Urticaria pigmentosa

        Review Date: 11/20/2012

        Reviewed By: Kevin Berman, MD, PhD, Atlanta Center for Dermatologic Disease, Atlanta, GA. Review provided by VeriMed Healthcare Network. Also reviewed by A.D.A.M. Health Solutions, Ebix, Inc., Editorial Team: David Zieve, MD, MHA, David R. Eltz, Stephanie Slon, and Nissi Wang.

        The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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