Mastocytosis

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:

• Detailed information about the causes of Mastocytosis including medication causes and drug interaction causes can be found in our causes pages.
• It is not known what causes mastocytosis, but a genetic problem has been found in some patients
• We do know some things that trigger the release of histamine from mast cells and cause the symptoms of mastocytosis.
• Mastocytosis probably is a hyperplastic response to an abnormal stimulus.
• It is not contagious, i.e. can't be caught by other people in contact with your child.
• Underlying medical conditions are other medical conditions that may possibly cause Mastocytosis.

Symptoms of Mastocytosis

Some sign and symptoms related to Mastocytosis:

• The mast cells do not usually cause problems. 4 out of 10 children with mastocytosis do not have symptoms.
• Anaphylaxis ( shock from allergic or immune causes)
• Stomach ulcers
• Episodes of very low blood pressure (including shock ) and faintness
• Their blood pressure may suddenly drop to a low level, causing them to faint.
• Abdominal discomfort
• Very rarely, there can be a strong reaction, with lightheadedness, breathing difficulty, wheezing and even fainting.

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.

• Antihistamines to work against mast cell chemicals
• Medicines to relieve cramping in the intestines

• Itching and other skin reactions
• Ulcer-like symptoms
• Low blood pressure
• Inability to take up nutrients from food

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.

Prevention Tips

• Cautious administration of aspirin to inhibit prostaglandin synthesis may be beneficial for patients with disease resistant to H1 and H2 antagonist therapy alone.
• Proton pump inhibitors help reduce production of gastric acid, which is often increased in patients with mastocytosis. Excess gastric acid can harm the stomach, esophagus, and small intestine.
• Corticosteroids can be used topically, inhaled, or systemically to reduce inflammation associated with mastocytosis
• Ultraviolet light and corticosteroid creams applied to the skin may be used to treat the skin symptoms of mastocytosis.
• Depression and other neurological symptoms have been noted in mastocytosis (see Rogers et al. ). Some antidepressants such as doxepin are themselves potent antihistamines and can help relieve physical as well as cognitive symptoms.
• Confirm the diagnosis of mastocytosis.
• Determine the extent of the condition. 

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