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Systemic Mastocytosis
Medical Questions » Systemic Mastocytosis
| Name: Systemic Mastocytosis |
| Also known as: |
| Form of urticaria pigmentosa that arises in an adult and affects internal organs as well as the skin. |
Causes of Systemic Mastocytosis
Skin and internal collections (patches) of mast cells that contain histamine. When the patch is disturbed, histamine is released into the tissue, and makes blood vessels leak, resulting in localized itching, swelling, redness and damage specific to the affected organ. |
Symptoms of Systemic Mastocytosis
Brown patches on the skin that steadily increase in number over several months or years. They blister and become itchy when rubbed. Internal patches may cause fever, weight loss, diarrhoea and abdominal pain. |
Tests for Systemic Mastocytosis
A skin biopsy can confirm the diagnosis of skin disease. The internal disease can be diagnosed by X-rays of the skull and long bones that may show areas of bone thinning. Liver function blood tests are only sometimes abnormal, and urinary histamine levels may be high. |
Treatment for Systemic Mastocytosis
Unsatisfactory, but antihistamines, steroids and interferon may be tried.
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Complications of Systemic Mastocytosis of its treatment
Rarely associated with cancer of the bone, liver, spleen, lymph nodes or gut, and causes a type of leukemia. |
Likely Outcome of Systemic Mastocytosis
Tends to persist long term. |
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