Mastocytosis: forms, symptoms, diagnosis, prognosis, treatment methods

August 15, 2017 23:00 | Allergies

Disease, when occurs in the tissues of the accumulation of mast cells and their proliferation, called mastocytosis.Such a phenomenon can take many forms and, depending on it, specialists determine the measures a patient needs to help.

Features disease

Manifestations of pathology are somewhat different in pediatric patients and in adults.

  • Children have a skin type of disease.It occurs more often at the age of up to two years.According to statistics, for 500 children there is one case of the disease.This kind of disease, in most cases, passes by itself and does not need special treatment.
  • 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)

Mastocytosis in children

forms mastocytosis

As manifestations of the disease and age-ill people distinguish these forms:

  • Cutaneous mastocytosis:
    • Infant shape - sick children up to two years.The signs of mastocytosis gradually go away.
    • Teenagers and people adulthood disease occurs:
      • Skin lesions are accompanied by signs of systemic lesions.However, the last symptoms do not go away, and do not progress.
      • The same, but there are already lesions of organs( manifestations of systemic mastocytosis).
  • systemic forms:
    • benign nature of the lesion organs,
    • manifestation of mastocytosis as a malignant form( mast cell leukemia).

Video below tells what mastocytosis( urticaria pigmentosa) Skin:

classification of skin lesions

skin lesions classified thus:

  • diffuse form - expressed largeFoci in the area of ​​the buttock folds and underarms.The lesions have a brownish-yellow color and cause a person's anxiety through itching. Changed skin areas create a patch of irregular shape, dense, to the touch, the 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 formations .With this form of skin lesions, there are spots of brownish-red color.If they are rubbed, then the spots become bumps.Their color is usually red.
  • Teleagioectasis form - this lesion consists of brownish-red spots.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.
  • Node 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 considered.

Symptoms

When skin form of the disease is characterized by such symptoms:

  • appearance on the skin of spots, papules or knots;
  • itching and discomfort at the site of rashes,
  • color of spots or other manifestations - pale pink, maybe with a yellow tint, there is a bright color - burgundy;
  • body temperature periodically rises above normal,
  • disease is accompanied by palpitations,
  • pressure tends to decrease.

If a systemic lesion is present, it means that 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.

Signs that are inherent in the system 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,
  • , neural tissue lesions are observed,
  • , negative processes in the digestive tract organs,
  • , spleen enlargement occurs.

Causes of

Mastocytosis 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.

Diagnostics

  • 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.
    • The content of mast cells or products of their vital activity is determined.
    • A special blood test determines the percentage of chromosome breakage.
  • For a more accurate diagnosis of the internal organs, it is necessary to be examined with instruments.Do:
    • computer diagnostics,
    • ultrasound examination,
    • endoscopy studies,
    • diagnosis by magnetic tomograph.

This video tells about the home therapy of mastocytosis, tells who and how it was cured:

Treatment

For assistance with mastocytosis, you need to contact a dermatologist.Self-medication is unacceptable.Children's mastocytosis can pass by itself.

The doctor's appointment takes into account the lesions that the patient has received.The specialist prescribes the patient symptomatic treatment.

Therapeutic way

For the treatment of manifestations of mastocytosis, physiotherapeutic procedures may be prescribed, namely, the use of ultraviolet irradiation.

By medicamentous method

Pigmentary-urticaria-1 For the relief of feeling 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

Systemic skin damage 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 with mastocytosis is shown in this video: