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Mastocytosis - Symptoms and Treatment

Mastocytosis 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. Mast cells are found in everyone in the skin, gut (stomach) and air passages. Mast cells contain many different natural chemicals, a common one being histamine. A number of things can trigger or cause the mast cells to release these chemicals, including heat, rubbing and certain foods and drugs. In children with mastocytosis they release more chemicals than their body needs due to the extra mast cells. The chemicals can cause symptoms that range from very minor to severe. There are three main forms of mastocytosis. In a rare form, mast cells accumulate as a single mass in the skin (mastocytoma). 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. Rarely, urticaria pigmentosa progresses to systemic mastocytosis during adulthood. In systemic mastocytosis, mast cells accumulate in the skin, stomach, intestines, liver, spleen, lymph nodes, and bones.

Mastocytosis is a disorder characterized by mast cell proliferation and accumulation within various organs, most commonly the skin. 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. 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. Although the majority of cases follow an indolent course, some patients may have evidence of a blood disorder such as a myelodysplastic or myeloproliferative disorder at the time of diagnosis. The course and prognosis of mastocytosis in these patients are determined by this associated hematologic disorder. More aggressive forms of mastocytosis and mast cell leukemias are very rarely encountered.

Causes of Mastocytosis

Common Causes of Mastocytosis :

  • Most cutaneous mastocytoses appear during infancy and early childhood. The risk of developing mastocytosis also increases during middle age.
  • The family history of mastocytosis, may also increase the chances of mastocytosis
  • Any abnormality to the blood, may lead to the condition of mastocytosis.

Symptoms of Mastocytosis

Some common Symptoms of Mastocytosis :

  • Nausea and vomiting.
  • Abdominal discomfort.
  • Blister.
  • Hives.
  • Low blood pressure.
  • Faintness.
  • Diarrhea.
  • Skin lesions .
  • Difficulty breathing.
  • Ulcers.

Treatment of Mastocytosis

  • Surgical removal of isolated mastocytomas, topical corticosteroid therapy and therapy with psoralen plus ultraviolet A (PUVA) to fade multiple pigmented lesions.
  • Antihistamines that works against ulcers and proton pump inhibitors relieve ulcer-like symptoms and also help to treat itching and other skin complaints.
  • It can be used topically, inhaled, or systemically to reduce inflammation associated with mastocytosis.
  • Malabsorption can be treated with steroids.
  • They are an important and often overlooked tool in the treatment of mastocytosis.
  • Two types of antihistamines treat severe flushing and low blood pressure before symptoms appear and epinephrine after symptoms begin .
  • Hot baths,Drinking hot liquids, Exercise,stress.








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