Learn About 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.
Mastocytosis; Maculopapular cutaneous mastocytosis; Diffuse cutaneous mastocystosis
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.
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
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.
Melody Carter is an Allergy and Immunologist and a Pediatrics provider in Bethesda, Maryland. Dr. Carter is rated as an Elite provider by MediFind in the treatment of Urticaria Pigmentosa. Her top areas of expertise are Mast Cell Activation Syndrome (MCAS), Urticaria Pigmentosa, Systemic Mastocytosis, and Anaphylaxis.
Regents Of The University Of Michigan
Cem Akin is an Allergy and Immunologist in Ann Arbor, Michigan. Dr. Akin is rated as an Elite provider by MediFind in the treatment of Urticaria Pigmentosa. His top areas of expertise are Systemic Mastocytosis, Mast Cell Activation Syndrome (MCAS), Anaphylaxis, Tissue Biopsy, and Septoplasty. Dr. Akin is currently accepting new patients.
Antonio Torrelo practices in Nino Jesus, Spain. Mr. Torrelo is rated as an Elite expert by MediFind in the treatment of Urticaria Pigmentosa. His top areas of expertise are Urticaria Pigmentosa, Impetigo, Atopic Dermatitis, and Perniosis.
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.
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.
Contact your provider if you notice symptoms of urticaria pigmentosa.
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.
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.