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Urticaria pigmentosa

Mastocytosis; Mastocytoma

 

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.

Causes

 

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
  • Sunlight, exposure to cold
  • Drugs, such as aspirin or other NSAIDs, codeine, morphine, x-ray dye, some anesthesia drugs, alcohol

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

 

Symptoms

 

The main symptom is brownish patches on the skin. These patches contain histamine. When histamine is triggered, 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
  • Rapid heartbeat

 

Exams and Tests

 

The health care provider will examine the skin. The provider may suspect urticarial pigmentosa when the skin patches are rubbed and raised bumps (hives) develop. This is called the Darier sign.

Tests to check for this condition are:

  • Skin biopsy to look for a higher number of mast cells
  • Urine histamine
  • Blood tests for blood cell counts and blood tryptase levels (tryptase is an enzyme found in mast cells)

 

Treatment

 

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.

 

Outlook (Prognosis)

 

Urticaria pigmentosa goes away by puberty in about 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.

 

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.

Bee 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 to Contact a Medical Professional

 

Call your provider if you notice symptoms of urticaria pigmentosa.

 

 

References

Habif TP. Urticaria, angioedema, and pruritus. In: Habif TP, ed. Clinical Dermatology: A Color Guide to Diagnosis and Therapy. 6th ed. Philadelphia, PA: Elsevier; 2016:chap 6.

Paller AS, Mancini AJ. Cutaneous tumors and tumor syndromes. In: Paller AS, Mancini AJ, eds. Hurwitz Clinical Pediatric Dermatology. 5th ed. Philadelphia, PA: Elsevier; 2016:chap 9.

 
  • Urticaria pigmentosa in the armpit

    Urticaria pigmentosa in the armpit - illustration

    Urticaria pigmentosa is characterized by few to many brownish spots which itch and, when scratched, produce welts and reddened skin. The brown spots contain cells with high levels of histamine. Individuals sometimes develop flushing and headache resulting from the massive release of histamine from these spots. This is the typical appearance of one of these spots (often referred to as a mastocytoma).

    Urticaria pigmentosa in the armpit

    illustration

  • Mastocytosis - diffuse cutaneous

    Mastocytosis - diffuse cutaneous - illustration

    This is a picture of diffuse, cutaneous mastocytosis. Abnormal collections of cells in the skin (mast cells) produce this rash. Unlike bullous mastocytosis, rubbing will not lead to formation of blisters (bullae).

    Mastocytosis - diffuse cutaneous

    illustration

  • Urticaria pigmentosa on the chest

    Urticaria pigmentosa on the chest - illustration

    Urticaria pigmentosa is characterized by several to many brownish spots that itch and, when scratched, produce welts and reddened skin. The brown spots, seen here on the chest, contain mast cells with high levels of histamine. People sometimes develop generalized flushing and headaches from the massive histamine release from these spots.

    Urticaria pigmentosa on the chest

    illustration

  • Urticaria pigmentosa - close-up

    Urticaria pigmentosa - close-up - illustration

    Urticaria pigmentosa is characterized by several to many brownish spots that itch and, when scratched, produce welts and reddened skin. The brown spots contain mast cells with high levels of histamine. People sometimes develop generalized flushing and headaches from the massive histamine release from these spots.

    Urticaria pigmentosa - close-up

    illustration

    • Urticaria pigmentosa in the armpit

      Urticaria pigmentosa in the armpit - illustration

      Urticaria pigmentosa is characterized by few to many brownish spots which itch and, when scratched, produce welts and reddened skin. The brown spots contain cells with high levels of histamine. Individuals sometimes develop flushing and headache resulting from the massive release of histamine from these spots. This is the typical appearance of one of these spots (often referred to as a mastocytoma).

      Urticaria pigmentosa in the armpit

      illustration

    • Mastocytosis - diffuse cutaneous

      Mastocytosis - diffuse cutaneous - illustration

      This is a picture of diffuse, cutaneous mastocytosis. Abnormal collections of cells in the skin (mast cells) produce this rash. Unlike bullous mastocytosis, rubbing will not lead to formation of blisters (bullae).

      Mastocytosis - diffuse cutaneous

      illustration

    • Urticaria pigmentosa on the chest

      Urticaria pigmentosa on the chest - illustration

      Urticaria pigmentosa is characterized by several to many brownish spots that itch and, when scratched, produce welts and reddened skin. The brown spots, seen here on the chest, contain mast cells with high levels of histamine. People sometimes develop generalized flushing and headaches from the massive histamine release from these spots.

      Urticaria pigmentosa on the chest

      illustration

    • Urticaria pigmentosa - close-up

      Urticaria pigmentosa - close-up - illustration

      Urticaria pigmentosa is characterized by several to many brownish spots that itch and, when scratched, produce welts and reddened skin. The brown spots contain mast cells with high levels of histamine. People sometimes develop generalized flushing and headaches from the massive histamine release from these spots.

      Urticaria pigmentosa - close-up

      illustration

    Self Care

     

       

      Review Date: 10/31/2016

      Reviewed By: Kevin Berman, MD, PhD, Atlanta Center for Dermatologic Disease, Atlanta, GA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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