Learn About Urticaria Pigmentosa

What is the definition of Urticaria Pigmentosa?

Urticaria pigmentosa is a skin disease that produces patches of darker skin and very bad itching. Hives can develop when these skin areas are rubbed.

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What are the alternative names for Urticaria Pigmentosa?

Mastocytosis; Maculopapular cutaneous mastocytosis; Diffuse cutaneous mastocystosis

What are the causes of Urticaria Pigmentosa?

Urticaria pigmentosa occurs when there are too many inflammatory cells (mast cells) in the skin. Mast cells are immune system cells that help the body fight infections. Mast cells make and release histamine, which causes nearby tissues to become swollen and inflamed.

Things that can trigger histamine release and skin symptoms include:

  • Rubbing the skin
  • Infections
  • Exercise
  • Drinking hot liquids, eating spicy food
  • Temperature changes, such as heat or cold
  • Stress or anxiety
  • Medicines, such as aspirin or other NSAIDs, codeine, morphine, x-ray dye, some anesthesia medicines, and alcohol

Urticaria pigmentosa is most common in children. It can also occur in adults.

What are the symptoms of Urticaria Pigmentosa?

The main symptom is brownish patches on the skin. These patches contain cells called mastocytes. When mastocytes release the chemical histamine, the patches develop into hive-like bumps. Younger children may develop a blister that is filled with fluid if the bump is scratched.

The face may also get red quickly.

In severe cases, these symptoms may occur:

  • Diarrhea
  • Fainting (uncommon)
  • Headache
  • Wheeze
  • Rapid heartbeat
  • Abdominal pain
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What are the current treatments for Urticaria Pigmentosa?

Avoid histamine release triggers.Triggers include insect bites and stings, exposure to temperature extremes, certain medicines, and rubbing the skin lesions.

Antihistamine medicines can help relieve symptoms such as itching and flushing. Talk to your provider about which type of antihistamine to use. Corticosteroids applied on the skin and light therapy can also be used in some cases.

Your provider may prescribe other kinds of medicine to treat symptoms of severe and unusual forms of urticaria pigmentosa.

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What is the outlook (prognosis) for Urticaria Pigmentosa?

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

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

What are the possible complications of Urticaria Pigmentosa?

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

Insect stings may also cause a bad allergic reaction in people with urticaria pigmentosa. Ask your provider if you should carry an epinephrine kit to use if you get a bee sting.

When should I contact a medical professional for Urticaria Pigmentosa?

Contact your provider if you notice symptoms of urticaria pigmentosa.

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Who are the sources who wrote this article ?

Published Date: November 30, 2022
Published By: Ramin Fathi, MD, FAAD, Director, Phoenix Surgical Dermatology Group, Phoenix, AZ. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

What are the references for this article ?

Dinulos JGH. Urticaria, angioedema, and pruritus. In: Dinulos JGH, ed. Habif's Clinical Dermatology. 7th ed. Philadelphia, PA: Elsevier; 2021:chap 6.

George TI, Sotlar K, Valent P, Horny H-P. Mastocytosis. In: Jaffe ES, Arber DA, Campo E, Harris NL, Quintanilla-Martinez L, eds. Hematopathology. 2nd ed. Philadelphia, PA: Elsevier; 2017:chap 49.

Wakelin SH. Dermatology. In: Feather A, Randall D, Waterhouse M, eds. Kumar and Clarke's Clinical Medicine. 10th ed. Philadelphia, PA: Elsevier Limited; 2021:chap 22.