Mastocytosis

Mastocytosis are a disorder characterized by mast cell proliferation and accumulation within various organs, particularly the skin. Mastectomies can occur in both children and adults.

Mast cells play an important role in helping the person’s immune system to defined their tissues from disease. Mast cells also provide immune defense system to the areas of the body where they are needed and they do this by releasing chemical alarms such as histamine and chytokines.

Mastocytosis is infact caused by the presence of too many mast cells in our body. Mast cells have other role also. Like they are seen to be gathered around the wounds and hence they play a major part in wound healing. For example, the particular itching, which a person feel around a healing scab, is caused by histamine released by the mast cells. Mast cells also have a role in the growth of blood vessels.

Symptms of Mastocytosis

Persons suffering from Mastocytosis may exhibit coetaneous lesions or systemic symptoms, which may be of acute nature or chronic nature. Most of the patients have pruritic cutaneous lesions.

Chemicals released by the mast cells causes changes in the body’s functioning that is it leads to specific allergic responses such as flushing, itching, abdominal cramping and even shock in certain cases. In presence of too many mast cells in the body, the additional chemical leads to Abdominal discomfort, ulcers, etc.

In case of the involvement of skeletal system, bone pain may be new onset of a fracture. Some patients, suffering from extensive cutaneous disease, may show acute symptoms associated with ingestion of certain drugs or foods. Symptoms such as headache, dyspnea , wheezing, rhinorrhoea, nausea, vomiting, diarrhoea and synocope may be noted.

When there is involvement of the Central nervous system then it leads to neuropsychiatric symptoms and few nonspecific changes such as malaise and irritability. Gastro intestinal involvement leads to weight loss. Mastocytosis sufferers also show tachycardia, hypotension, anorexia, etc.

Mastocytosis affects males and females equally. Mostly patients with mastocytosis are children. Many cases of Mastocytosis are seen in whites. The cutaneous lesions of majority of mastocytosis are less visible of more heavily pigmented skin.

Associated systemic symptoms of mastocytosis are considered to be due to the release of mast cell derived mediates, such as histamine, heparin, prostaglandins, neutral proteases and acid hydrolases. The skeletal, gastrointestinal, cardiopulmonary, haemopoietic and central nervous systems may be involved either directly via mast cell infiltration or indirectly via mast cell mediator release.

Treatment for Mastocytosis

Antihistamines are commonly used to prevent mastocytosis. Antihistamines generally treat itching and other skin complaints. Some antihistamines work specifically against ulcers and proton pump relief to ulcer like symptoms.

Antihistamines also treat severe flushing and low blood pressure before the symptoms appear. Topical steroids may be used in case of mastocytosis or they temporarily reduce skin lesions that are cosmetically disturbing. Steroids also help in treatment of malabsorption or decreased ability of individual to take in nutrients.

Anticholinergic drugs are used to provide relief from Intestinal cramping. In case, if the mastocytosis is malignant, cancerous or associated with a blood disorder then chemotherapy is generally given. Mastocytosis is usually diagnosed by skin biopsy and search for specific genetic mutation is done.

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