Mastocytosis develops when mast cells increase in number and accumulate in tissues over a period of years. Systemic mastocytosis, often termed systemic mast cell disease (SMCD), is characterized by mast cell infiltration of extracutaneous organs, which is in contrast to cutaneous mast cell disorders, which involve only the skin. In very rare cases, mastocytosis can affect other parts of the body, like the stomach, the intestines and the bone marrow. Mastocytosis is listed as a " rare disease " by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH) There are three main forms of mastocytosis. In a rare form, mast cells accumulate as a single mass in the skin (mastocytoma).
Mastocytosis is rare. It differs from typical allergic reactions because it is chronic rather than episodic. The more common form of the disease is known as Urticaria Pigmentosa (UP), the name used when there is an increase of mast cells in the skin. This condition results when a person has too many mast cells. Typically, a mastocytoma develops before age 6 months. In a form called urticaria pigmentosa, mast cells accumulate in many areas of the skin, forming small reddish brown spots or bumps.The more serious form of the disease is known as Systemic Mastocytosis (SM), meaning the mast cells are increased in other areas of the body.
Cause of Mastocytosis
Here are the list of the possible cause of Mastocytosis:
Symptoms of Mastocytosis
Some sign and symptoms related to Mastocytosis:
Classification of Mastocytosis - systemic and diffuse
The presence of too many mast cells, or mastocytosis , can occur in two forms - cutaneous (skin) and systemic (involves internal organs).
Cutaneous mastocytosis (CM), the most common form, occurs when mast cells increase in the skin. It is also called urticaria pigmentosa. CM mostly affects children.
Systemic mastocytosis is caused when mast cells collect in the tissues and can affect organs such as the liver , spleen , lymph nodes , and bone marrow.
Diffuse Mastocytosis is the most comon form of Mastocytosis
Diagnosis of Mastocytosis
Doctors can diagnose mastocytosis or urticaria pigmentosa by seeing the characteristic lesions which are dark-brown and fixed. A small skin sample may help confirm the diagnosis of mastocytosis disorder.
Treatment of Mastocytosis
Several medicines help treat the symptoms of mastocytosis.
In rare cases in which mastocytosis or uticaria is cancerous or associated with a blood disorder, the patient may have to use steroids and/or chemotherapy.
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