Mast cells are produced in the bone marrow. Mast cells play an important role in the immune system, protecting tissues from infection and parasites – mainly the skin, the mucous membranes of the respiratory system, and the digestive tract. Mast cells produce various substances, so-called "mediators", during an allergic reaction. One of these substances is histamine, which can cause itching, redness and swelling of the skin; headaches; dizziness; nausea; and diarrhea.

Both children and adults can suffer from mastocytosis.

There are two types of mastocytosis: cutaneous mastocytosis (where the skin is affected) and systemic mastcytosis (which affects inner organts).

This form of mastocytosis is characterized by skin lesions; inner organs are not affected. This form of mastocytosis generally appears first in early childhood and is relatively benign.

Systemic mastocytosis means that the inner organs are affected by the proliferation of mast cells. In some cases the skin is also affected.

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