Basic Pediatric Mastocytosis Information Sheet

Mastocytosis is a rare disease. The cause is unknown and there is no cure. A child with mastocytosis has an abnormal accumulation of mast cells. This may occur in the skin or almost any tissue in the body.

Mast cells are located normally in nearly all tissues in the body, with their concentration in the GI tract, the skin, and the lining of the respiratory system. Mast cells are involved in tissue repair and growth, control of the flow of blood into areas where it is needed, protecting the body against infection, and communicating with other cells of the immune system. Mast cells contain little packets or granules of chemicals. When these are released, the cell is said to "degranulate." This sends a message to the tissue and blood to alert immune system cells of jobs that need to be done.

Mast cells include many chemicals, including histamine. When mast cells degranulate this causes allergic-type symptoms for a mastocytosis patient. There are several types of mastocytosis, generally manifesting itself in the skin as small tan, red-brown to yellow lesions, or in a raised or flat reddish-brown area, or over-all doughy orange-peel looking skin, to "normal" looking skin- depending on the type of mastocytosis. Outward appearance is not always an indication of mast cell stability. Mastocytosis may have adverse effects on the interior of the body, as well. The common side effects of mastocytosis include headache, gut pain, and bone pain. It is important to know that this disease is not contagious!

This disease may follow a very mild course or may be accompanied by severe complications. Common triggers for children with mastocytosis are change in temperature, friction on the skin, and emotional stress. Other triggers include certain drugs/medications, foods, dyes/preservatives, and insect stings.

Reactions may include all or some of these listed; hives, blisters, itching, and flushing. If triggered a child may also experience immediate stomach symptoms, change in mood, and even anaphylaxis.

Children with mastocytosis are generally treated with anti-histamines daily and/or as needed. They may also take several other kinds of medication to control reactions in the skin and internally. It is important for mastocytosis patients to try to avoid triggers known to that particular individual. Although, it may not be common for children to experience anaphylactic shock, it must be known that the possiblility is there for even the mildest cases. Especially if exposed to severe temperature change, such as a fall into a cold pool. Bee or wasp stings are also known to be a possible cause of anaphylaxis for mastocytosis patients.

Note: Information for each individual child including specific type of mastocytosis, triggers, reactions, instructions for medicating etc., and medication/epi-pen should accompany this document to be given to care-givers (teacher, school nurse, principal, day care provider, etc.)