Mastocytosis - Causes, Symptoms and Treatment
The abnormal agglomeration of mast cells in the skin and sometimes in various other parts of the body, is refered to mastocytosis. Mast cells are cells of the immune system that are found around blood vessels in the skin, gastrointestinal tract, respiratory tract, and genitourinary tract. They release chemicals including histamine that are very irritating and cause itching, swelling, and fluid leakage from cells. Mastocytosis is a disease characterized by the presence of too many mast cells in various organs and tissues.
It is a condition characterized by the abnormal proliferation of mast cells in the tissues. 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.It is not contagious, i.e. can't be caught by other people in contact with your child.
Mastocytosis comes in three forms. The three forms are:
- Mastocytoma, a benign skin tumor.
- Urticaria pigmentosa, small collections of mast cells in the skin that manifest as salmon or brown-colored patches.
- Systemic mastocytosis, the collection of mast cells in the skin, lymph nodes, liver, spleen, gastrointestinal tract, and bones.
Causes of mastocytosis:-
There is no known cause of mastocytosis. However, the following factors can raise a person's risk of developing mastocytosis:
- Cancer:- Cancer is main cause of mastocytosis. A person , who is suffering from skin cancer, are more prone to get affected by mastocytosis.
- Blood disorders:- Any abnormality to the blood, may lead to the condition of mastocytosis.
- Age. Most cutaneous mastocytoses appear during infancy and early childhood. The risk of developing mastocytosis also increases during middle age.
- C-kit receptor mutation. The c-kit receptor is a protein on the surface of some cells that binds to stem cell factor. A mutation of c-kit may cause mastocytosis.
- Genetic: The family history of mastocytosis, may also increase the chances of mastocytosis.
Symptoms of mastocytosis:-
Some important symptoms of mastocytosis includes:-
- Allergy:- It includes: itching , abdominal cramping.
- Occasionally even blister can form.
- You will have have the difficulty in breathing, in case you are affected by mastocytosis.
- There will be many pink or brown spots on the skin, which may be flat or raised. The spots may be anywhere on the body, but especially on the trunk and limbs.
- Abdominal discomfort is common in mastocytosis.
- Episodes of very low blood pressure and faintness
- Nausea and vomiting may occur in chronic condition.
- During mastocytosis, there will be pain in bone or muscle.
- If there are many spots on the skin then it can cause flushing, irregular heart beat, tiredness and headaches.
- Diarrhoea and tummy pain are possible symptoms.
Treatment of mastocytosis:-
Some main treatments for mastocytosis includes:
- Histamine H 2 blockers can be used to decrease the gastric hyperacidity that often accompanies concomitant ulcer disease in patients with systemic mastocytosis.
- Orally administered cromolyn sodium can relieve diarrhea and abdominal pain.
- Corticosteroids: It can be used topically, inhaled, or systemically to reduce inflammation associated with mastocytosis.
- Immunotherapy: It may be beneficial for the treatment mastocytosis. It reduces the rashes from the skin.
- Surgical removal of isolated mastocytomas, topical corticosteroid therapy and therapy with psoralen plus ultraviolet A (PUVA) to fade multiple pigmented lesions.
- Aspirin: It directly triggers mast cell degranulation.
- Malabsorption can be treated with steroids.
- Pharmacologic treatment of mastocytosis involves stabilizing mast cell membranes by blocking the action of inflammatory mediators.
- Antidepressants: They are an important and often overlooked tool in the treatment of mastocytosis.