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Mastocytosis

What is Mastocytosis?

Mastocytosis (mas-toe-sigh-toe-sis) is a disorder caused by having too many mast cells in a person's body. Mast cells are a kind of blood cell that is located in the skin, the lining of the stomach, connective tissue (such as cartilage or tendons) and intestines. Mast cells are important for survival. They help defend the skin, stomach, and intestines against diseases. Mast cells are also involved in healing wounds.

Mastocytosis can occur at any age. However, it's usually more serious in adults. Mastocytosis is usually mild in children and they often outgrow it.

Mastocytosis was originally described by E. Nettleship and W. Tay. Nettleship and Tay described it as a "Rare form of Urticaria" in the British Medical Journal, 1869. In 1877 tissue mast cells were identified by Paul Ehrlich. In 1949, an autopsy by J.M. Ellis proved that mastocytosis involved the internal organs.

Types of Mastocytosis

There are two types of mastocytosis: cutaneous (skin) and systemic. There are different types of cutaneous and systemic forms. The most common cutaneous form is called urticaria pigmentosa. Urticaria pigmentosa occurs when mast cells get into the skin. Urticaria pigmentosa was first reported in the scientific literature in 1933.

Systemic mastocytosis is caused by the accumulation of mast cells in the tissues. It can affect organs such as the liver, spleen, bone marrow, and small intestine.

What are Mast Cells?

Mast cells are a type of cell that helps you fight off infections. They make a chemical called histamine. Histamine can cause swelling, itching, and redness when your body is reacting to something. There are more mast cells in the skin, the lungs and the intestines than in other parts of your body.

What Causes Mastocytosis?

The exact cause of mastocytosis is unknown.

What are the Symptoms of Mastocytosis?

The symptoms of mastocytosis vary between individuals. The symptoms are different, depending on where the extra mast cells are. Symptoms of mastocytosis in the skin are a red and itchy rash, hives, and a rash that looks like freckles, or a lump on your skin. Symptoms of mastocytosis in the stomach and intestine are diarrhea and stomach pain.

Other symptoms of mastocytosis include abdominal cramping, bone pain, abdominal discomfort, nausea and vomiting, ulcers, diarrhea, skin lesions, and episodes of hypotension or shock.

How does will the doctor detect mastocytosis?

The doctor can detect mastocytosis by performing a skin biopsy, a bone marrow biopsy, a blood test, or a urine test.

Can mastocytosis be treated?

Mastocytosis can be treated. Your doctor can prescribe medication to alleviate the symptoms. Unfortunately, mastocytosis cannot be cured.

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