Disease, when occurs in the tissues of the accumulation of mast cells and their proliferation, called mastocytosis. This phenomenon can take many forms and, depending on this, specialists determine the measures a patient needs to help.
Features of the disease
Manifestations of pathology are somewhat different in patients of childhood and adults.
- Children have a skin type of disease. It occurs more often at the age of up to two years. According to statistics, 500 children have one case of the disease. This kind of disease, in most cases, passes by itself and does not need special treatment.
- The adult form is also a rare disease. The statistics show that one out of a thousand people falls ill. But only this form of the disease does not usually pass by itself.
The disease is more severe than the sight of more children. Systemic mastocytosis damages internal organs.
Mastocytosis in children( photo)
Forms of mastocytosis
According to the manifestations of the disease and the age of the sick people, the following forms are distinguished:
- Skin form of mastocytosis:
- Infant form - children under 2 years of age. The signs of mastocytosis gradually go away.
- Adolescents and adults of the adult period have a disease:
- Skin lesions are accompanied by signs of systemic lesions. However, the last symptoms do not go away, and they do not progress.
- The same, but there are already lesions of the organs( manifestations of systemic mastocytosis).
- Systemic form:
- is a lesion of benign nature,
- manifestation of mastocytosis as a malignant form( mast cell leukemia).
The video below will tell you what the mastocytosis( pigmentary urticaria) of the skin is:
Classification for skin lesions
Skin lesions are classified as follows:
- The diffuse form of is expressed by large foci in the area of the buttock folds and underarms. The lesions have a brownish-yellow color and cause a person's anxiety through the itch. Changed skin areas create a patch of irregularly shaped, dense, to the touch, constitution. Their boundaries are clearly delineated. The surface of the hearth can be damaged, cracked. With minor injuries, blisters can appear on the surface of the lesion. The process is typical to regress with time.
- Macular Papular Diseases .With this form of skin lesions, spots of brownish-red color are observed. If they are rubbed, then the spots become bumps. Their color is usually red.
- Teleagioectasis form - this lesion consists of spots of brownish-red color. They are subcutaneous vessels in the dilated state. The area where the spots are located is sharply pigmented. From the friction of the lesion can be covered with blisters. The process is able to develop and hit the bones. Mastocytosis of this form is found mainly in women.
- Mastocytoma - this form of lesion appears as separate nodes. Their size can be in the diameter of 2 ÷ 5 cm. Their surface looks like an orange peel or is quite smooth.
- Nodal type - a large number of nodules are defined in the cutaneous layer. They have a spherical shape and dimensions within 1 cm. The surface of the tubercle is flat, the consistence is dense. The color of the formations is yellow, red, pink.
Next, the diagnosis, symptoms, causes and treatment of mastocytosis in adults and children will be examined.
Symptoms of
The cutaneous form of the disease is characterized by the following symptoms:
- appearance on the skin of spots, papules or knots;
- pruritus and discomfort at the site of rashes,
- color of spots or other manifestations - pale pink, maybe with a yellow tinge, there is a bright color - burgundy;
- body temperature periodically rises above normal,
- disease is accompanied by palpitations,
- pressure tends to decrease.
If there is a systemic lesion, it means that the symptoms that are characteristic of the cutaneous form may also appear. This is possible if systemic disorders are combined with a cutaneous manifestation of the disease.
Symptoms inherent in the systemic form:
- in the bone marrow, the process of substitution of healthy cells for mast cells,
- bone system is subjected to destructive phenomena, osteoporosis and osteosclerosis are observed, the patient suffers from bone pain;
- in liver tissues local replacement of liver cells occurs on cells of connective tissue,
- there are lesions of nervous tissue,
- negative processes in the organs of the digestive tract,
- there is an increase in the spleen.
Causes of
Medical science has not yet guessed why a small number of people have this disease. There are suggestions that pathology is associated with a genetic predisposition. However, in practice, the theory is not fully confirmed, because predispositions do not show up in half of people who have fallen ill.
About the symptoms that has skin and systemic mastocytosis, read below.
Diagnosis
- To determine if a patient has a mastocytosis disease, external manifestations on the skin are examined and examined( if any).Also, when viewed, the condition of the internal organs can be checked.
- A biopsy of skin samples taken from the lesion site is made. If necessary, a large number of mast cells are biopsied and organs( bone marrow).
- To understand whether there is a disease or not, it is necessary to perform a blood test.
- Determines the content of mast cells or products of their vital functions.
- A special blood test determines the percentage of chromosome breakage.
- For more accurate diagnosis of the internal organs, it is necessary to be inspected with instruments. Do:
- computer diagnostics,
- ultrasound examination,
- endoscopic examinations,
- diagnosis by magnetic tomograph.
This video tells about the home therapy of mastocytosis, tells who and how it was cured:
Treatment of
For assistance with mastocytosis, it is necessary to consult a dermatologist. Self-medication is unacceptable. Children's mastocytosis is able to pass by itself.
The doctor's appointment takes into account the lesions that the patient received. The specialist prescribes the patient symptomatic treatment.
Therapeutic method
For the treatment of manifestations of mastocytosis, physiotherapeutic procedures may be prescribed, namely, the use of ultraviolet irradiation.
By medicamentous method
For the relief of health in case of skin problems, a specialist may prescribe the use of( external) corticosteroid preparations.
In cases of both systemic lesion and skin variant, antihistamines are used.
Operation
Surgical intervention is prescribed by a specialist in cases where the formations on the skin have increased to large sizes.
In systemic lesions, it is sometimes prescribed to remove organs if specialists find it necessary.
Prevention of the disease
To exclude the provocation of the disease, it is necessary to remove the factors that contribute to the increase in mast cells.
This:
- cold,
- insolation,
- trauma,
- use of medications that help increase the number of mast cells.
Complications of
Systemic damage to the skin can lead to serious organ damage. This situation significantly affects their ability to perform their functions.
Forecast
- The development of events is expected with a positive outlook. Children often have problems themselves.
- With a systemic lesion, the prognosis is very different. In the case of mast cell leukemia, we can talk about the unfavorable course of events.
ultrasound in case of mastocytosis is shown in this video: