Mast Cell Disease (Urticaria Pigmentosa)

Your child has been diagnosed with urticaria pigmentosa (UP).  These skin spots often start as a few red bumps that increase in number with time.  They appear most often on the trunk, but the face, arms, and legs can be involved as well. Over time, the areas may look brown instead of red.

 

These areas are caused by the build-up of too many mast cells in the skin.  A mast cell is a cell of the immune system that holds histamine and other chemicals that can affect the skin.  If the skin that contains the mast cells is stroked or scratched, the histamine is released.  This causes redness and swelling there; or a “hive.”  Sometimes, the child may have blisters where the skin was scratched or stroked.

 

UP often begins in the first 8 months of life.  During the next few months, more bumps may appear.  By age 5, the lesions do not flare and “hive” with scratching.  Most of the time, you will notice the bumps but there are no symptoms.  By teen years, most lesions are either gone or have only faint areas of brown pigment left.

 

There are medicines that can cause the mast cells to release histamine and cause symptoms throughout the body, such as flushing, rapid heart rate, low blood pressure with fainting, vomiting, and diarrhea.

 

Triggers for Mast Cell Disease

 

Items on this list are most often not a problem unless the mast cell disease is severe.  Exposures should be reduced in severe cases of mastocytosis.

 

Physical stimuli:  Exercise, heat, skin friction, hot baths, hot drinks, cold exposure (especially swimming), sunlight, emotional stress, spicy foods.

 

Medicines, oral and injected:  Aspirin, alcohol (in cough compounds and pediatric elixers), morphine, codeine, dextromethorphan (“DM” in cough compounds), nonsteroidal anti-inflammatory agents, procaine, opiates (codeine, Demerol, morphine), thiamine, high molecular weight polymers (dextran).

 

Medicines, topical:  Topical antibiotics containing polymyxin B.

 

Medicines sometimes used with general anesthesia:  D-tubocurarine, scopolamine, decamethonium, resperpine.

 

X-Ray Contrasts and Agents:  Radiographic dyes, gallamine.

 

Venoms:  Snakebites, bee stings, jellyfish stings.

 

Histamine Containing Foods:  Parmesan, Blue, and Roquefort cheeses, spinach, eggplant, some red wine, tuna, mackeral, bonita, skipjack.

 

Histamine Releasing Foods:  Ethanol, egg white, crustaceans (crayfish, lobsters), chocolate, strawberries, tomatoes, citrus.

 

Treatment

 

Your doctor may prescribe a strong medicated cream or ointment that will decrease the number of mast cells in the skin.  You should apply this medicine to each red/brown area on your child’s skin twice a day for 10 days.  Then stop for 10 days.  If the skin spots are still present, repeat the treatment twice a day for 10 more days, and then stop.

 

Your child may also be prescribed a histamine-blocking medicine to take by mouth.  This should be taken every day as prescribed by the doctor, not just when your child has symptoms.  This prevents much of the redness, swelling in the skin, and itching that comes from the excess histamine released from the mast cells.  

 

UP may bother your child because it is noticeable on the skin and can be itchy. But it is benign and goes away with time.  If you have further questions, please call the Pediatric Dermatology clinic.

 

Joyce M. C. Teng, M.D., PhD

 

UW Dermatology Department

1 S. Park St 7th Floor

Madison, WI 53715

Clinic: 608 287-2450

 

American Family Children’s Hospital

Pediatric Dermatology Specialty Clinic

1675 Highland Ave.

Madison, WI  53792

Clinic: 608 263-6420

 

The information provided should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Any duplication or distribution of the information contained herein is strictly prohibited.

Last Updated: 06/17/2009

Copyright © 05/01/2009 University of Wisconsin Hospitals and Clinics Authority. All rights reserved. Produced by the Department of Nursing. UWH #6454

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