Mastocytosis - Definition, Causes, Symptoms and Treatment

Definition:-

Mastocytosis is characterized by infiltration of mast cells into the tissues of the body. Mast cells are connective tissue cells which release chemicals including histamine that are very irritating and cause itching, swelling, and fluid leakage from cells. This disease is a group of rare disorders of both children and adults caused by the presence of too many mast cells in a person's body.

The general form of mastocytosis is a disease of the skin. Rarely mastocytosis affects other parts of the body, like the intestines,stomach and the bone marrow. This condition results because of too many mast cells in person's body.. Mastocytosis can occur in any age. It's more serious in adults and mild in children.

The body has a variety of free-roaming cell populations that function as immunogenic agents. Most immunogenic cells fall into the category of white blood cells, but some remain in tissues and are not found in the blood. Mast cells are such a group. Mast cells play an important role in helping your immune system defend these tissues from disease. Mast cells also attract other key players of the immune defense system to areas of your body where they are needed by releasing chemical agents such as histamine and cytokines.

Symptoms:-

The possible symptoms of mastocytosis are as:-

• The occurance of red and itchy rashes over the skin.
• You may have the pain over musculoskeletal.
• Hives
• Abdominal discomfort
• Difficulty breathing
• Nausea and vomiting are common during mastocytosis.
• Ulcers
• Stomach pain
• Diarrhea
• Bone pain
• Skin lesions
• Disease can cause episodes of hypotension (very low blood pressure and faintness) or anaphylaxis (shock).

Causes:-

The exact cause of mastocytosis is uncertain, but there are some factors that triggers the mastocytosis. These includes:-

• Bone or muscle pain.
• Abdominal discomfort.
• Nausea and vomiting.
• Stomach ulcers.
• Diarrhea.
• Skin lesions.
• Episodes of very low blood pressure and faintness.
• Age: cutaneous mastocytoses generally appears during infancy and early childhood. The risk of developing of the disease also increases during middle age.
• C-kit receptor mutation:The c-kit receptor is a type of protein generally found on the surface of some cells that binds to stem cell factor. A mutation of c-kit may cause some types of mastocytosis.
• In some cases of Mastocytosis, it can also be caused by genetic problem .

Treatment:

The effective treatments for mastocytosis are as:-

• Doctors recommend medicines to treat mastocytosis which are antihistamines (to prevent the effect of mast cell histamine) and anticholinergics.
• Antihistamines that works against ulcers and proton pump inhibitors relieve ulcer-like symptoms and also help to treat itching and other skin complaints.
• Mast cell stabilizers help prevent mast cells from releasing their chemical contents.
• Corticosteroids can be used topically, inhaled, or systemically to reduce inflammation associated with mastocytosis.
• Two types of antihistamines treat severe flushing and low blood pressure before symptoms appear and epinephrine after symptoms begin .
• Steroids treat malabsorption, or impaired ability to take in nutrients and reduces skin lesions that are cosmetically disturbin
• In children, a mastocytoma generaly disappears spontaneously. Itching due to urticaria pigmentosa may be treated with antihistamines.
• Things to avoid: Hot baths,Drinking hot liquids, Exercise,stress.

Medicines that stimulate Mastocytosis and should be avoided by patients:

• Aspirin 
• Narcotics
• Reserpine
• Iodine-containing contrast for X-rays