Urticaria Pigmentosa
Symptoms, Doctors, Treatments, Advances & More

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.

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
  • Wheezing
  • 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.

Who are the top Urticaria Pigmentosa Local Doctors?
Elite in Urticaria Pigmentosa
Elite in Urticaria Pigmentosa

Molecular Medicine And Neuroscience

10 Center Dr Bldg 10, 
Bethesda, MD 
Languages Spoken:
English

Melody Carter is a Pediatrics provider practicing medicine in Bethesda, Maryland. Dr. Carter is rated as an Elite provider by MediFind in the treatment of Urticaria Pigmentosa. She is also highly rated in 7 other conditions, according to our data. Her clinical expertise encompasses Mast Cell Activation Syndrome (MCAS), Urticaria Pigmentosa, Systemic Mastocytosis, and Anaphylaxis.

Elite in Urticaria Pigmentosa
Internal Medicine
Elite in Urticaria Pigmentosa
Internal Medicine

Domino's Farms

24 Frank Lloyd Wright Dr Ste 2100, Lobby H, 
Ann Arbor, MI 
Languages Spoken:
English
Offers Telehealth

Dr. Akin completed his residency in Internal Medicine and PhD at the University of Louisville, and his Allergy/Immunology fellowship at the NIH Clinical Center, where he also worked as a staff physician and researcher. He was then an Assistant Professor at the University of Michigan and an Associate Professor at Harvard Medical School, where he established and lead the Mastocytosis Center at the Brigham and Women's Hospital in Boston. He is internationally recognized for his work on mast cell disorders and anaphylaxis and is an active member of the AAAAI. Dr. Akin is rated as an Elite provider by MediFind in the treatment of Urticaria Pigmentosa. He is also highly rated in 20 other conditions, according to our data. His clinical expertise encompasses Systemic Mastocytosis, Mast Cell Activation Syndrome (MCAS), Anaphylaxis, Urticaria Pigmentosa, and Tissue Biopsy.

 
 
 
 
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Elite in Urticaria Pigmentosa
Elite in Urticaria Pigmentosa
Nino Jesus, MD, ES 

Antonio Torrelo practices practicing medicine in Nino Jesus, Spain. Mr. Torrelo is rated as an Elite expert by MediFind in the treatment of Urticaria Pigmentosa. He is also highly rated in 25 other conditions, according to our data. His clinical expertise encompasses Urticaria Pigmentosa, Impetigo, Perniosis, and Atopic Dermatitis.

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: October 14, 2024
Published By: Elika Hoss, MD, Assistant Professor of Dermatology, Mayo Clinic, Scottsdale, 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 ?

Akin C. Mastocytosis. In: Goldman L, Cooney KA, eds. Goldman-Cecil Medicine. 27th ed. Philadelphia, PA: Elsevier; 2024:chap 235.

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

Tzankov A, Sotlar K, Valent P, Horny H-P, George TI. Mastocytosis. In: Jaffe ES, Arber DA, Campo E, et al, eds. Hematopathology. 3rd ed. Philadelphia, PA: Elsevier; 2025:chap 49.