Kids Health Info

Mastocytosis

  • Mastocytosis (urticaria pigmentosa) is a mostly harmless condition where your child has more than the normal number of mast cells in the body. Mast cells are found in everyone in the skin, bowels, gut (stomach), lungs and air passages.

    Mast cells do not usually cause problems. In fact, they play an important role in helping our body's immune system to protect the body against diseases.

    A person with mastocytosis in the skin will have pink or brown flat spots that will eventually go away after several years. Some people will have only a few of these spots while others will have many.

    Who can get mastocytosis?

    Mastocytosis can come on at any age, but the disease is different when adults get it. Children with mastocytosis usually get it in their first year of life and will usually have an increase in the number of mast cells in the skin only. It is very rare for children to have an increase in mast cells in other organs.

    In children who have mastocytosis in the skin only, it is most likely that it will go away by itself.  However, this may take many years. Seven out of 10 children with mastocytosis in their skin can expect a major improvement by the time they are 10 years old.

    Signs and symptoms

    Mast cells contain many different natural chemicals, a common one being histamine. A number of things can cause the mast cells to release these chemicals including heat, rubbing and certain foods and drugs. In children with mastocytosis, the mast cells release more chemicals than their body needs because they have extra mast cells. The chemicals can cause symptoms that range from very minor to severe.

    • The mast cells do not usually cause problems. Four out of 10 children with mastocytosis do not have symptoms.
    • The skin will have one or many pink or brown spots, which may be flat or raised. The spots may be anywhere on the body, but are most commonly found on the trunk and limbs. As the condition gets better, the spots often fade; but they may remain for years.
    • When the mast cells in the skin release their chemicals, it can cause the spots to become itchy, red and swollen (like hives); and occasionally the spots may even form blisters. This may happen for no reason, but is more common after stroking, rubbing or exposure to heat.
    • If there are only one or two spots, the chemicals released by the mast cells are not enough to cause other problems.
    • If there are many spots on the skin, other possible symptoms can include flushing, irregular heart beat, tiredness, headaches, diarrhoea, tummy pain, ulcers, nausea and vomiting. 
    • Very rarely, strong reactions such as light-headedness, breathing difficulties, wheezing and even fainting may occur.

    Causes

    • It is not known what causes mastocytosis, but a genetic problem has been found in some patients.
    • It is not usually passed on from one generation to the next.
    • It is not contagious, i.e. it can't be caught by other people in contact with your child.

    Treatment

    Sometimes a blood test and/or a skin test is required. This can:

    • confirm the diagnosis of mastocytosis
    • determine the extent of the condition
    • help to predict the course of the condition.

    Often no medication is needed. However, antihistamines (e.g. Zyrtec, Claratyne, Zantac) in tablet or syrup form may be used to relieve symptoms including the itching. You can buy these from your local chemist. Topical cortisone creams and wet wraps have also been found to be helpful.

    We do not recommend the routine carrying of an Epi-pen.

    Care at home

    Care at home involves avoiding the things that trigger or cause a response in your child.

    Medications

    Some medications can stimulate mast cells to release histamine. It is better that your child avoids these. For example, pain killers, cough medicines and anaesthetics can cause problems. Always check medications with your child's doctor. If your child is having surgery, inform the anaesthetist about the mastocytosis.

    The list of medicines to avoid includes:
     Aspirin  Scopolamine
     Non-steroidal anti-inflammatories  Gallium
     Decamethonium  Quinine
     Narcotics  Polymyxin B
     Iodine-containing contrast for X-rays  Amphotericin
     Beta-blockers  d-Tubocurarine
     Reserpine  Any drug that has previously caused a reaction

    Food

    Some children find that large amounts of certain foods can also cause a flare up of the condition. The following is a list of foods that have been found to cause problems for some children:
     Alcohol  Cola  Nuts   Spinach
     Aubergine/Eggplant  Egg White  Onion  Strawberry
     Avocado  Fermented foods  Paprika  Tomato
     Banana  Grains  Pork  Tuna 
     Beans (boiled)  Herring  Pineapple  Cheese
     Fruit juices  Salami  Citrus fruits  Mackerel
     Salmon  Cocoa  Maize  Shellfish
     Coconut  Millet  Soybeans  

    Other things that may trigger a response are:

    • hot baths
    • drinking hot liquids
    • swimming in cold water
    • exercise
    • emotion or stress. 

    Follow up

    • Your child should have regular check ups  with their family doctor or dermatologist if they have more than a few spots.
    • Your child's growth and development should also be checked regularly, however there are usually no problems.

    Please contact your child's doctor if they have problems such as:

    • skin flushing, diarrhoea, headaches or bone aches that continue
    • if they look pale and unwell.

    Key points to remember

    • Mastocytosis in children is usually a harmless condition that goes away over some years.
    • It can't be caught by others and is not usually passed on from generation to the next.
    • Adult mastocytosis is different to childhood mastocytosis.

    Other sources of information

    • Ask your child's dermatologist or paediatrician if you would like any further information. Please be aware that information about adult mastocytosis may not be relevant to children with mastocytosis.
    • Support group website: www.mastokids.org
    • Kids Health Info factsheet: Find a doctor

     

     

    Developed by the Department of Dermatology. First developed 2004. Updated November 2010.


Disclaimer 
This information is intended to support, not replace, discussion with your doctor or healthcare professionals. The authors of these consumer health information handouts have made a considerable effort to ensure the information is accurate, up to date and easy to understand. The Royal Children's Hospital, Melbourne accepts no responsibility for any inaccuracies, information perceived as misleading, or the success of any treatment regimen detailed in these handouts. Information contained in the handouts is updated regularly and therefore you should always check you are referring to the most recent version of the handout. The onus is on you, the user, to ensure that you have downloaded the most up-to-date version of a consumer health information handout.