I got this information sheet from my pedi dermatologist with guidelines about Urticaria Pigmentosa. I thought I would type up this information in case other doctors haven't given it out.
What is Urticaria Pigmentosa (UP)?
Urticaria Pigmentosa (urr-tah-care-ee-ah pig-men-toes-ah) is a rash that affects the neck, arms, legs, and trunk of infants, children and young adults. This kind of rash does not happen very often. The rash is made up of red to brown color spots that are flat or slightly raised. Sometimes the spots will blister. There may be a few spots or many spots. The spots form hives when the rash is rubbed or scratched.
What causes Urticaria Pigmentosa
UP happens when there is an increased number of mast cells in the skin. The exact cause for the increased number of mast cells is not known. Mast cells are present in almost all parts of the body and help to fight infection. They also make a substance called histamine. Histamine causes hives, itching, flushing (redness) and the red color of the rash of UP. Many things may trigger UP. A trigger is something that results in the rash and itchy skin of UP. To understand what a trigger is, think of a light switch that turns on and off. A trigger is like a light switch. Things that turn "on" the skin rash of UP are called triggers.
How is Urticaria Pigmentosa diagnosed?
Urticaria Pigmentosa is diagnosed by signs and symptoms. The skin rash of UP and the itching help the doctor make the diagnosis.
What tests might my child have?
A skin biopsy can be done but may not be necessary. Sometimes other studies such as x-rays and blood tests are done as well.
How is Urticaria Pigmentosa treated?
Use medicine as directed: The medicine may make the rash less itchy. This medicine is given by mouth and is called an anti-histamine. An anti-histamine is a medicine used to decrease the release of the histamine from mast cells.
Take care of your child's skin: Sun exposure may increase the brown color of the rash. Applying a sunscreen with SPF 15 may help to prevent the spots from becoming darker. If the rash becomes dry, scaly or rough, apply a moisturizing cream or ointment 3 to 4 times a day.
Avoid things that trigger UP: Triggers do not cause UP but they may bring out the rash or make the rash worse. In addition, massive release of histamine in the skin can cause low blood pressure or even shock. You will need to avoid things that trigger the release of histamine.
How to avoid things that trigger Urticaria Pigmentosa
There are a number of things that can trigger the rash of UP. Some of the triggers include emotional stress, medicines, foods and physical stimuli.
Foods and Liquids: Such as cheese, lobster, jelly fish, crayfish, spicy foods, and drinking hot liquids.
Drugs: Some of these medicines are used in the home. Other medicines are only used in the doctor's office or in the hospital: aspirin, codeine, morphine, alcohol, polymyxin B (found in polysporin ointment), thiamine (a vitamin), quinine, dyes that are used to take x-rays, opiates, nonsteroidal anti-inflammatory agents, D-tubocurarine, scopolamine, procaine, gallamine, decamethonium, pancurinium, and dextromethorphan.
Physical Stimuli: are mechanical or external factors that cause more histamine to be released: exercise, vigorous rubbing of the skin after bathing or showering, hot baths, drinking hot liquids, spicy foods, sunlight and cold exposure (especially swimming).
Other: Emotional stress, IV high molecular weight polymers (compound 48/80, dextran), calcium inonophor A23187, snake venom, Hymenoptera venoms, complement-derived anaphylotoxins, bacterial toxins, adenosine triphosphate (ATP).
How does Urticaria Pigmentosa affect your child?
The rash of UP is not painful but does cause itching in some children. Massive release of histamine can cause headaches, flushing (redness of this skin), diarrhea, vomitting, wheezing (breathing hard with a whistling sound), increased heart rate and a decrease in blood pressure.
How long does Urticaria Pigmentosa last?
Most infants and children will outgrow UP as they get older. New spots may continue to appear as long as the condition lasts. There is no known treatment to prevent new spots from appearing. The reddish-brown spots may fade as your child grows older but can last for months or even years.
This information sheet also recommends ordering a medical bracelet for your child to wear that reads: Urticaria Pigmentosa (Mastocytosis). Your child should wear this bracelet at all times. If your child has surgery or goes to the emergency room, make sure to provide them with this information because a lot of the triggers are used in emergency situations.
Alert - Take your child to the Emergency Room if your child has:
Wheezing or fainting
Problems with breathing
Call your child's doctor, nurse or clinic if you have any questions or concerns. Call right away if your child has:
Diarrhea that does not go away.
I hope this information is helpful to anyone who's child has UP.