Paediatric Mastocytosis

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Paediatric Factsheet

Millie's Story

Cutaneous Mastocytosis

Triggers and Degranulation

Histamine Rich Foods

Kidz Bitz

 

Paediatric Mastocytosis can be a very distressing condition, both for the child and for its parents.  Very young children often cannot describe their symptoms of pain and discomfort, and because of this, and the rarity of the disease, diagnosis may be delayed.  Fortunately, this form of mastocytosis often resolves itself in adolescence or adulthood.  Those children who do retain the condition into adulthood usually have a milder form of the disease, and it is unlikely to develop into systemic mastocytosis.

The condition usually manifests itself in one of two forms of Cutaneous Mastocytosis - Urticaria Pigmentosa or Diffuse Cutaneous Mastocytosis.  Triggers for both types are the same as for adult systemic mastocytosis - stress, friction on the skin, temperature change and a number of drugs, medications, food dyes, preservatives and insect stings.

Urticaria Pigmentosa manifests itself as red/brown spots, either flat or raised which are usually small and over all of the body, but can be larger.  The trunk is most commonly affected, and the palms and soles of the feet are not generally involved.  These spots can also form hives when rubbed.  The spots can also form blisters, but these are rarely seen after two years of age.

Diffuse Cutaneous mastocytosis is the more rare of the two forms, in which large numbers of mast cells infiltrate the skin, giving rise to round blisters and thickened, leathery skin.  These lesions generally form a wheal or hive if rubbed.  This is known as Darier's sign.  The blisters are sterile when formed, but can become infected if the child rubs or scratches.  The hive is caused by the release of histamine and other potent chemicals from degranulating mast cells.  These chemicals can also cause wheezing, diahorea, low blood pressure and flushing.

 

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Parents may have a particularly difficult time when their children start to attend school or nursery.  Other children can be cruel and insensitive.

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Millies Story.