12 August

Cutaneous mastocytosis - types , symptoms , treatments

Contents:

1. causes of mastocytosis

2. Types of mastocytosis

3. symptoms, diagnosis and treatment of mastocytosis

Mastocytosis - is a complex systemic disease, whichdue to the accumulation and proliferation of mast cells in the tissues of the body.The disease (a malignant form is called a mast cell leukemia) was first described in 1869 as chronic urticaria, leaves behind a brownish stain.The term mastocytosis was introduced only in 1953.It is a rare disease, which is much more common in children, and, as a rule, children mastocytosis limited lesion of the skin, while the adult are almost always develops systemic mastocytosis.

mastocytosis most commonly affects the skin of children, but also may affect other organs, such as intestines, stomach, spleen, liver, lymph nodes or bone.More severe cases of mast cell leukemia accompanied by such a severe blood diseases like acute leukemia, chronic neutropenia, or lymphoma.Approximately 90% of patients with mastocytosis, urticaria pigmentosa

suffer.Treatment of mastocytosis often is to assign different antihistamines and control of major diseases.

causes of mastocytosis

etiology of the disease is unknown.Mast cells are distributed in almost all organs and are located in close proximity to the lymphatic and blood vessels, epithelial surfaces and peripheral nerves, which allows them to carry out protective and regulatory functions, as well as participate in a variety of inflammatory reactions.

pathogenesis of the disease based on the release of various mediators of mast cells, including heparin, histamine, leukotrienes, cytokines and inflammation.An extensive body of infiltration by mast cells, leads to violations of its functions.Among the reasons, which are able to launch the selection of mediators, say heavy exercise, the effect of various toxic substances of chemical or organic origin.Also noted in the etiology of the disease mutation of genes that encode receptor tyrosine kinases.There is a theory about the transmission of the disease in an autosomal dominant way, although most of the patients have no family history.

Types of mastocytosis

basis of clinical features, are four forms of the disease:

  • Cutaneous mastocytosis infants often occurs in children in the first two years of life.A distinctive feature of this type of disease manifestations, is the complete absence of any lesions in the internal organs.All cutaneous manifestations usually disappear completely at puberty and never appear.Cutaneous mastocytosis in children flowing with pronounced symptoms, requires the timely and proper treatment.
  • Cutaneous mastocytosis adolescents and adults is characterized by abundant infiltration of mast cells not only skin, but also the various internal organs.However, in contrast to more complex forms of systemic illness, resulting in this form of the disease, the changes do not progress in the internal organs.Only in some cases, cutaneous mastocytosis can move in systemic form.
  • Systemic mastocytosis generally develops in adults and is the progressive loss of various internal organs in combination with skin changes.This form of the disease found only in 10% of cases and usually develops, following cutaneous manifestations.
  • mast cell leukemia - a malignant form of mastocytosis, which is caused by degeneration of fat cells.Infiltrates of mast cells modified extensively affect the internal organs and tissues, which leads to death.In this form of the disease cutaneous manifestations usually completely absent.

symptoms, diagnosis and treatment of mastocytosis

When cutaneous form of the disease mainly affects the skin and highlights the main symptoms such as itchy skin, heart attacks, skin redness, pressure reduction and intermittent fever.

Cutaneous mastocytosis is characterized by five main types of skin lesions:

  • maculopapular type - characterized by the occurrence of multiple reddish-brown spots with a strong pigmentation and clear outlines boundaries.When mechanical stress, spots turn into red bumps, resembling hives;
  • node type - manifests itself in the appearance of the skin tissues of multiple nodular seals up to 1 cm in diameter.This seals the color can be red, yellow or pink, and nodules may coalesce into large plaques;
  • solitary form - manifested form large mastocytoma, up to 5 cm thick, rubber-like consistency.Most often only one generation, but are also found to form a shape with four nodes.Solitary shape characterizes only mastocytosis in infants;
  • erythrodermic form of mastocytosis is characterized by clearly defined yellow-brown lesions dense consistency, with jagged edges.Also for this type is characterized by severe pruritus.Most often localized rashes in the armpits and gluteal fossa.Even slight mechanical injury foci leads to increased itching and blistering;
  • teleangiektaticheskaya form - is characterized by patches of varying size, red-brown color, arranged, usually in the chest and limbs skin.When a mechanical force on the ground spots appear blisters, itching is possible, the process may spread to the bone.This form of the disease occurs mainly in adults.

If systemic mastocytosis various internal organs mast cells infiltrate.At the same time the diseased organs, there are multiple specific disorders - bone pain, induration and enlargement of the liver, diarrhea, ulcerative lesions of the gastrointestinal tract, enlargement of the spleen, the formation of leukemia, swollen lymph nodes, nervous tissue damage.

basis for diagnosis of mastocytosis are the clinical manifestations of the disease and on the detection of skin and tissues of a large number of fat cells.Apply complex laboratory and instrumental methods of investigation - blood tests, skin biopsy of the affected organs and bone marrow, a chromosomal analysis to identify anomalies, X-rays of bones, MRI, ultrasound and CT scans of internal organs, endoscopic methods of diagnosis.

If mastocytosis in children is not accompanied by any systemic symptoms, the treatment he needs, because it has a great tendency to heal itself through adolescence.Diseases are hematologists and physicians, specific treatments for mastocytosis today is not, therefore, apply symptomatic therapy.Treatment of mastocytosis is based largely on the use of antihistamines, which inhibit the production of biologically active substances by mast cells.When complex forms of mastocytosis, and severe disease shows the use of PUVA therapy.Surgery to remove the spleen as required under diffuse systemic mastocytosis.Forecast treat mastocytosis determined form of the disease and the degree of internal organ involvement.

Attention!

This article is available exclusively in the educational purposes and is not research material or professional medical advice.

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