Mastocytosis: Symptoms and Treatment
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Mastocytosis called systemic, current chronic disease caused by the proliferation of mast cells (mast cells). This is a rare systemic disease usually occurs in children during the first three years of life often goes alone to the period of puberty.
The causes and mechanisms of development of the disease are not well understood. Theorizes inheritance in an autosomal dominant pattern, but the exact mechanism is not known.
The main symptoms of mastocytosis depends on its shape. There are:
- cutaneous mastocytosis,
- dermal-visceral or systemic mastocytosis.
Skin lesions may have several different forms:
- nodosum occurs mainly in young children. The skin plaques appear yellow to 2 cm in diameter with a smooth surface or similar to orange peel.
- bullous to form bubbles to 2 cm in diameter and with a clear content.
- diffuse, occurs in adults and in children. The skin is affected on large areas. Items with sharp boundaries. Skin yellow, thick or soft, similar to orange peel.
- mottled papulleznaya, pigment, usually found in adults. It manifested located on the trunk brown spots or tubercles 5-7 mm. Eruptions can alternate with episodes of remission. When combing elements turn into blisters.
- On the mucous membranes and the skin may occur telangiectasia — dilated subcutaneous vessels.
Systemic mastocytosis is manifested in the skin appearance or all individual manifestations of cutaneous lesions to the liver and spleen, bone osteoporosis, swelling and itching of the skin, digestive disorders, tachycardia and pressure fluctuations, hemorrhagic syndrome, fever. Seizures occur due to mechanical friction skin or physical and chemical stimuli.
The most severe form of mastocytosis — mast cell leukemia, malignant mastocytosis mast cells with lesions of the internal organs.
Some forms of mastocytosis require only a dynamic monitoring and control. More complex is the question of how to treat systemic mastocytosis. Assign antiserotoninovym antihistamines and drugs, reserpine, photosensitizing drugs. In severe cases, hormones are used.