Urticaria pigmentosa mastocytosis. What is mastocytosis (urticaria pigmentosa) and what are the causes of the appearance in a child? Treatment and photo of children with a rash

Mastocytosis: what is it? Urticaria pigmentosa (English urticaria pigmentosa, ICD-10 code Q82.2 Mastocytosis L50.8 Other urticaria) is an allergic skin reaction that causes discoloration of the affected areas or, conversely, their darkening, as well as severe itching.

State caused by the presence too much a large number of mast cells(another name is mastocytes) in the skin. Their peculiar work is to inflame the foci of inflammation, they release a chemical called histamine in response to microbes and other viruses.

Mastocytosis (urticaria pigmentosa) is a condition in which too many mast cells accumulate in the skin.

This disease more often Total occurs in infants and children, although adults also sometimes note the development of mastocytosis.

Most children have urticaria pigmentosa goes away after puberty. The severe form usually occurs in older children or adults.

Sometimes urticaria pigmentosa can develop into systemic mastocytosis. With it, mast cells can accumulate in other organs of the body.

In some cases this can lead to mast cell leukemia or mast cell sarcoma, both of which are malignant.

Symptoms

The main symptom of mastocytosis is the appearance of reddish-brown itchy patches that look like freckles and/or bumps (papules) that appear on the skin. Rarely, lesions may also develop in the bones or other organs of the body (eg, bone marrow, esophagus).

When these spots are touched or exposed to heat, they become slightly convex and begin to turn red or white, which is very noticeable compared to the healthy tissues surrounding them.

This reaction is known as Darier's sign(used for diagnosis). These areas, often referred to as blisters or scars, tend to be very itchy and can change size and shape within hours.

Sometimes these blisters may become inflamed and swollen, in this case, patients note the occurrence of fluid inside them.

Other symptoms may include:

1. Hyperemia (reddening of the skin).
2. Changing the color of the spots to dark brown or black.
3. Severe diarrhea.
4. Tachycardia (rapid heartbeat).
5. Nausea or vomiting.
6. Fainting or fainting.
7. Dizziness and headache

Reference! Rarely, against the background of urticaria pigmentosa in adult patients, telangiectasia can develop - this is an expansion of the superficial vessels of the skin.

In most cases symptoms urticaria pigmentosa last for several days and then subside. Some patients notice traces of pigmented skin that remain for many years.

A more severe form of urticaria pigmentosa systemic mastocytosis proceeds with approximately the same symptoms, but sometimes the following are added to them:

• anaphylaxis;
• labored breathing;
• low blood pressure;
• cramps in the uterus, bleeding;
• musculoskeletal pain.

It should be noted that systemic mastocytosis, the symptoms mentioned above, are very rare in individuals diagnosed with urticaria pigmentosa or "systemic mastocytosis", but may occur with other forms of mastocytosis, such as aggressive systemic mastocytosis.

Causes

Exact reasons occurrence of urticaria pigmentosa not yet explored. In some cases, it occurs for no apparent reason, sometimes the specialist suggests that it was inherited as an autosomal dominant genetic trait. However, not all children with the defective gene will have manifestations of the disease.

Genetic diseases are determined by a combination of genes for a particular trait, which are located on the chromosomes received from the father and mother.

Attention! The abnormal gene can be inherited from one or both parents. Transfer risk of such a gene from sick parents to children is 50% for each pregnancy, regardless of the sex of the unborn child.

Urticaria pigmentosa (mastocytosis) most often occurs on the background too much a large number inflammatory cells ( mast cells) in the skin.

Factors that activate the increase mast cell concentrations:

1. Physical stimuli (heat, friction).
2. Excessive physical activity.
3. Bacterial toxins (as well as poisons).
4. Body poisoning.
5. Eye drops containing dextran.
6. Alcohol.
7. Some foods: lobster, crayfish, fish, cheese, lobster, very hot drinks, spicy food.
8. Abuse of painkillers.
9. Stress, depression.

Systemic mastocytosis may precede, but most often is a complication urticaria pigmentosa. The likelihood of developing systemic mastocytosis increases with age and approaches the mark in 15-30% in adults with urticaria pigmentosa.

Urticaria pigmentosa: photo

Systemic mastocytosis: photo

Diagnostics

The diagnosis of "urticaria pigmentosa" is made on the basis of a visual examination of the patient.

An allergist (or dermatologist) may test an area of ​​skin for the presence of mast cells. by friction. If dark red or brown rashes appear at the place of friction, then this indicates the presence of urticaria pigmentosa.

They may also be assigned tests how:

• skin biopsy to determine the total number of mast cells;
• analysis for histamine in the urine;
• a complete blood count to measure tryptase levels (tryptase is an enzyme found in mast cells);
• bone marrow biopsy;
• a skeletal bone scan (x-ray) can show areas of bone thinning.

Systemic mastocytosis is also diagnosed by bone marrow and skin biopsy.

Most common skin lesions due to urticaria pigmentosa look the same(by color and shape). Lesions that are different from others may be a sign of cancer.

Possible types of cancer:

• melanoma;
• basal cell carcinoma;
• senile keratosis (seborrheic).

Contacting a doctor with suspicion of urticaria pigmentosa inevitably.

An ambulance may be needed if:

1. The patient has difficulty breathing, wheezing is heard.
2. There was swelling of the throat or tongue.
3. The patient notes a constant feeling of weakness, there are symptoms such as high fever or chills.
4. There is a persistent feeling of nausea, vomiting or diarrhea.

Treatment

When a disease such as mastocytosis appears, treatment must be carried out under the supervision of a doctor.

First steps

1. Don't rub your skin and do not scratch the inflammation, even if it is very itchy.
2. Don't try to pop the blister or squeeze liquid out of it.
3. try reduce rash contact with clothing, wear only loose clothing.

Immediately stop taking medicines such as:

• aspirin (in small doses is not dangerous);
• codeine.

Important! Don't take a hot bath with urticaria pigmentosa, this will only worsen your condition.

Medical

Identifying and avoiding the causes of the disease may be sufficient to prevent symptoms of mild urticaria pigmentosa.

Mastocytosis in adults, drug treatment:

1. H1 antihistamines used to relieve symptoms such as itching and flushing, as well as H2 antihistamines- for the treatment of hyperacidity. To reduce the risk of anaphylactic shock, both H1 and H2 blockers are used.

   In rare but severe cases, the patient is given a medical bracelet that notifies the release of histamines, in which case the patient must inject himself with an injection of adrenaline (epinephrine).

   A similar course of action is necessary to avoid circulatory collapse and shock.

2. Mast cell stabilizers, such as sodium cromoglycate, promote degranulation of mast cells after exposure to specific antigens. These agents fight diarrhea, abdominal pain, headaches and bone pain well.

   The course of treatment with such drugs is usually about a week.

3. Small doses of aspirin may also help, although aggravation may occur in some cases. Treatment with small doses of aspirin is indicated patients with acute vascular insufficiency.
4. Photochemotherapy. If urticaria pigmentosa appears, the treatment is long-wave UVA radiation (340-400 nm). A decrease in the concentration of mast cells is noted in the irradiated skin. Course of treatment: 2-3 procedures every week for several months.

   Photochemotherapy reduces itching and improves the appearance of the skin.

5. Steroid drugs and creams used to treat systemic mastocytosis.
6. Recently, therapy with interferon and imatinib has been used to treat severe form, as well as to treat systemic mastocytosis ( antileukemic drug).

Examples of drugs are presented below.

Allergists often prescribe the drug Cromolyn 200 mg orally four times a day (100 mg four times a day for children 2 to 12 years of age).

Antihistamine Ketotifen 2 to 4 mg taken orally is also effective against urticaria pigmentosa.

Ketotifen's analogs: AllergoKomod, Dipolkrom, Kromoheksal.

Patients with an aggressive form of the disease are administered the drug Altevir(interferon alfa-2b) subcutaneously once a week, it causes regression of bone lesions. Corticosteroids(eg, prednisolone 40 to 60 mg orally once a day for 2 to 3 weeks) may also reduce symptoms.

Attention!Any of the above remedies should be started take only after consultation with your doctor (therapist, allergist, dermatologist).

Often performed for cosmetic reasons. removal of dark pigment spots. This is a very expensive but painless procedure. The price usually depends on the diameter of the stain, removing a stain of 1 cm in size costs about 2000 rubles.

Folk remedies

Urticaria pigmentosa in adults and children is also treated with folk remedies. Let's find out what will help get rid of the disease:

Diet

With urticaria pigmentosa you should follow the following rules:

1. Eliminate alcohol completely.
2. Eliminate spicy and fried foods from your diet.
3. Add organic oils such as coconut or olive oil to your diet.
4. Drink plenty of water, at least 1.5 liters per day.
5. Reduce your intake of saturated fats (dairy) and increase your intake of omega-3s (nuts, avocados).
6. Eat enough fiber (whole grain bread).
7. Reduce your intake of sugar and salt.
8. Eat fresh fruits and vegetables.
9. Don't neglect physical activity.

Important! Symptoms of urticaria pigmentosa in children disappear when they reach puberty (in about half of the cases). In adults urticaria pigmentosa can develop into systemic mastocytosis.

This is a serious disease that can affect various organs, in any case, self-treatment of the disease is not recommended.

For the types of cutaneous mastocytosis and the manifestation of the disease in men and women, see the following video:

Urticaria is a skin disease of allergic origin, the main symptoms of which are a small rash similar to blisters, constantly itchy.

One type of urticaria is urticaria pigmentosa.

Urticaria pigmentosa or mastocytosis is a disease in which mast cells accumulate in the blood, tissues, mainly the skin. In most cases, this disease appears in young children. Mastocytosis is a rare disease that occurs equally in men and women, but most often in children.

There are several types of mastocytosis:

1. Urticaria pigmentosa, pigmentary mastocytosis;
2. Papular mastocytosis;
3. nodular mastocytosis;
4. Multinodular mastocytosis;
5. Vesicular mastocytosis;
6. Tumor-like mastocytosis;
7. Diffuse, or erythrodermic mastocytosis;
8. Visceral mastocytosis;
9. Telangiectatic (or erythematous-spotted, eruptive) mastocytosis;
10. atrophic mastocytosis.

The disease can disturb a person both for a short time and for a long period of life.

You can make a small description of the individual forms of mastocytosis.

Skin form: manifested by the appearance of red spots, papules on the skin.

Persistent spotted telangiectotic form: Appears only in adults as freckles, but all over the body.

Diffuse mastocytosis: symptoms are yellow thickening of the skin; location: inguinal folds.
Nodular urticaria pigmentosa: appears in young children in the first month of their life.

Erythrodermic urticaria pigmentosa: a very rare form, in children it appears as blisters, in adults as red rashes.

Solitary urticaria pigmentosa: this form is characterized by the appearance of small nodules on which bubbles form. This form is the most severe of all urticaria, it only develops with age.

In medicine, benign and malignant mastocytosis are distinguished.

Benign is also divided into systemic mastocytosis and cutaneous mastocytosis.

The systemic form can occur without damage to the skin, but affects the internal organs. This is a form that can be inside the body, and a person will not even suspect it. And after puberty, the disease will manifest itself from the outside.

Systemic mastocytosis affects the internal organs of a person: most often bones, disorders of the organs of the gastrointestinal tract, liver, in some cases causes an ulcer, and also disrupts the composition of the blood.

Cutaneous mastocytosis affects the skin in the form of a rash.

The reasons

The causes of mastocytosis are not yet fully understood, but doctors refer to some factors:

• the genetic nature of the disease;
• inflammatory processes in the body, infectious diseases;
• under the influence of immune and non-immune factors.

Diagnostics

At the first examination of the patient, the doctor examines the skin, the affected areas.

After the examination, a biopsy begins - a research method in which several examples of cells and tissues are taken from the patient from the site of the skin lesion.

In laboratory studies, a change in indicators is detected: hypocholesterolemia, thrombocytopenia, eosinophilia.

Chromosomal analysis is carried out.

The bones are x-rayed.

An ultrasound of the liver and especially the spleen is done.

Symptoms

How exactly does urticaria pigmentosa manifest itself on the skin?

The main symptoms are: a rash on the skin of various types, severe itching, decreased pressure, frequent heartbeat, sometimes the temperature rises. This is due to the release of histamine from mast cells.

Mastocytosis mainly affects the limbs and torso, scalp, and oral mucosa. After a while, they can move to other areas of the skin.

Effect on internal organs

As mentioned above, mastocytosis affects not only the skin, but also the internal organs. How exactly do we understand now.

Liver- increases, fibrous nodes appear and it thickens, tissues change to connective.

Bones- there is an area of ​​osteoporosis - the bones soften, and osteosclerosis develops, naturally, they begin to hurt.

lymph nodes- increase and hurt.

Gastrointestinal system- develops peptic ulcer, diarrhea.

Spleen- greatly increases.

Treatment

Doctors are involved in the treatment: therapists, dermatologists and hematologists.

Specific treatment for this disease has not yet been developed, symptomatic treatment is used, due to which the production of biologically active substances by mast cells is suppressed.

Consider the algorithm for the treatment of urticaria pigmentosa:

Folk remedies

Baths for systemic mastocytosis

Ingredients: valerian root, celandine, medicinal sage, chamomile flowers, tripartite string, St. John's wort. Take the same amount. Boil for 10 minutes, then insist half an hour. We filter the decoction. The infusion is poured into the heated water in the bathroom. Bathe in such a bath for ten minutes for a couple of weeks.

Mastocytosis tincture

Ingredients: 1 tablespoon of nettle color, 1 cup of hot water. Infuse for half an hour. Strain. Drink half a glass 4-5 times a day, warm up a little before use.
A very good remedy in the fight against urticaria is freshly squeezed juice from odorous celery. Take half an hour before meals, 3-4 times a day.

Dry celery tincture

Ingredients: pour two tablespoons of raw materials with one liter of water, always cold, you need to insist for two hours. Drink half an hour before meals, three times a day, one-third of a glass.

Calamus Powder

Ingredients: Pour calamus root with warm water, and apply before going to bed, half a teaspoon.

Video: General understanding of mastocytosis

Urticaria pigmentosa is called mastocytosis. The disease occurs in young children. It also occurs in adults. The disease is associated with a violation of the pigmentation of the skin.

Urticaria pigmentosa is associated with a violation of skin pigmentation - hence the common name.

Experts do not name the causes of urticaria pigmentosa. They put forward two hypotheses. The first suggests that mastocytosis is hereditary. The theory is supported by the fact that the disease occurs in people who are closely related.

According to the second theory, mastocytosis occurs when the immune system fails. Patients facing inflammatory processes are more likely to suffer from urticaria pigmentosa. Mastocytosis also occurs for no apparent reason.

Urticaria is manifested by the accumulation of excess mast cells in the tissues. Their destruction leads to the release of histamine, heparin and other substances. They increase vascular permeability. Tissue edema develops. The capillaries expand. There is a change in the skin. Arterial pressure goes down. The body temperature rises, tachycardia develops. Symptoms are accompanied by severe itching.

A number of factors influence the increase in histamine:

• temperature changes;
• mechanical impact;
• exposure to solar radiation;
• changing of the climate;
• stress and nervous tension;
• toxic effect;
• allergic reaction;
• taking medication.

Urticaria pigmentosa or mastocytosis stains inflamed areas in a different color. The intensity of the shade depends on the deposition of melanin in the lower layer of the skin. Experts attribute this to the interaction of mast cells and melatocytes.

Symptoms of mastocytosis

Symptoms of hives include the formation of red or brown spots on the skin. These are areas with jagged edges. The formations resemble freckles, or papules. Areas rise above the surface of the skin. In severe form, the bones and internal organs are affected.

Under mechanical action, the pigment area protrudes and changes color. The change in shade is noticeable in comparison with healthy areas of the skin. The reaction is called Darier's symptom.

Another symptom of urticaria pigmentosa is itching. Burning leads to scratching of the blisters and their damage. Inflammations change shape, increase or disappear over time.

Dermatologists note reddening of the skin in patients. In some situations, inflammation becomes dark brown or black. Patients are prone to diarrhea, palpitations, nausea. There is weakness and faintness.

Symptoms persist for 2-3 days. After that, the signs of the disease disappear. Systemic mastocytosis is more severe. Patients have:

• allergic reaction;
• breathing problems;
• pressure drop;
• bleeding, convulsions;
• pain syndrome in bones and muscles.

Different forms of the disease are characterized by the listed symptoms. If one or more signs appear, a dermatologist's consultation is required.

How is mastocytosis in children

Mastocytosis in children often occurs in infancy. Urticaria in infancy is characterized by the formation of red and brown spots. The rash is accompanied by severe itching. Mechanical impact leads to the formation of blisters. After healing, pigmentation remains on the injured areas, which persists for several years.

Formations are localized on the body, legs and arms of the patient. Rarely, rashes form on the face. Brown nodules and plaques form on the affected areas.

The disease is not accompanied by malaise of the child. Fever, fever, generalized itching may occur. Urticaria pigmentosa in children does not progress to a systemic form.

The disease is diagnosed by a dermatologist. The conclusion is made on the basis of external examination and complaints. In some situations, scrapings are taken from the affected area. In parallel with the examination, the child undergoes an ultrasound of the internal organs and takes a detailed blood test. It is carried out to exclude the systemic form of urticaria. Treatment is symptomatic.

Forms and types of the disease

Until now, dermatologists have not determined the nature of mastocytosis. The disease manifests itself both in childhood and in adulthood. Family history is not confirmed. Dermatologists call 4 types of the disease.

1. Cutaneous mastocytosis in infancy. The rash appears in infancy. Reappearance is possible during puberty. The disease does not develop. There is no system component.
2. Urticaria with systemic changes. There are changes in the internal organs. The disease does not develop. In childhood, the pathology regresses. In older patients, the disease becomes systemic.
3. Systemic mastocytosis. Leads to damage to internal organs. It develops against the background of external signs of the disease.
4. Malignant mastocytosis. It is called mast cell leukemia. It is determined by the presence of malignant mast cells in organs, tissues and bone skeleton. There are no external signs of manifestation. Possible death.

Mastocytosis is also subdivided according to the type of skin manifestations.

1. node type. It passes with the formation of nodes and tubercles, with a smooth or bumpy surface. The color of formations is yellow, red, pink. The tone is different from healthy skin. The number of nodes varies by the degree of the disease. Rashes are combined into single areas.
2. Maculopapular type. It is characterized by the appearance of nodules of dark color with even contours. Mechanical action leads to the appearance of bubbles. Outwardly similar to hives.
3. Infant type. It is called solitary mastocytosis. Associated with the appearance of nodes, up to 50 mm in size. The skin on the knot is dense. Location of knots: neck, body, arms. In infants, predominantly one mastocytoma occurs. Rarely up to 4 pieces. Formations are characterized by a sharp appearance and disappearance.
4. erythrodermic type. It is characterized by the appearance of yellow or brown spots with a dense surface. The boundaries of inflammation are clearly defined, the edges are uneven. The disease is characterized by severe itching. As a result of combing, the inflamed areas are injured.
5. Telangiectatic type. Belongs to the category of persistent urticaria pigmentosa. Rarely seen in patients. It appears in adulthood in women. Characterized by spots of red and brown. The nodes are localized on the chest, legs and arms of the patient.

Features of diagnosis and treatment

Mastocytosis in adults and children is diagnosed by a dermatologist. Therapy is selected for each case separately. Treatment is prescribed depending on the age and type of disease. Mild cases of urticaria do not require therapy. The disease heals on its own.

In severe situations, symptomatic therapy is prescribed:

• taking antihistamines (Diazolin, Tavegil, Zodak, Zirtek, Suprastin);
• the use of antiserotonin agents (Ketotifen, Bikarfen);
• corticosteroids, creams and ointments for severe blisters and nodules;
• products with cromoglyceric acid;
• histaglobulin.

PUVA therapy is actively used for mastocytosis. The skin is irradiated with ultraviolet rays. The length of the rays is fixed. The patient is taking medication at the same time. Drugs contribute to the penetration of radiation deep into the epidermis. Surgical intervention is used in case of ineffective conservative treatment.

At all stages of treatment, patients comply with dietary nutrition. Patients exclude food allergens, fried, salted and smoked. Do not use products with dyes and flavors. The skin is protected from mechanical stress and temperature changes.

Infantile mastocytosis resolves by puberty. In adulthood, cutaneous mastocytosis progresses. The disease affects the internal organs, leading to death.

Urticaria pigmentosa is a type of skin disease known as mastocytosis. In general, mastocytosis has nothing to do with true urticaria. The disease begins with the fact that in the living tissues of the body gradually accumulate, and then actively multiply mast cells. They play an important role in the formation of the immune system in the body. Therefore, with urticaria pigmentosa, immunity is weakened.

This type of disease most often affects young children. However, adults can also get sick. In this case, usually the urticaria that has arisen in a child passes quite quickly with proper treatment. But an adult disease can accompany the whole life, periodically appearing, then disappearing.

Scientists have not yet fully studied this disease. Therefore, it is impossible to clearly identify certain causes provoking it. But still there are factors in which mastocytosis develops in about 70% of all cases. These are infectious and genetic factors.

Let's consider them in more detail:

1. People have a genetic predisposition to mastocytosis if close blood relatives also suffered from this disease during their lives. As a rule, the disease begins to manifest itself when some physical factors coincide. It can be severe cold, heat, which constantly affect the body, friction or squeezing. In the event that some parts of the body are constantly exposed to such insignificant, at first glance, factors, then there is a risk of developing urticaria pigmentosa.
2. Very often, urticaria develops in a person if he spends too much time in direct sunlight. Ultraviolet helps to change the structure of certain components of cells. As a result, this affects both the general well-being and the skin.
3. Stressful situations and frequent climate changes are external factors that contribute to the development of mastocytosis. As a rule, it harms the human psyche, disrupts the normal functioning of organs. As a result, the immune system is also weakened, and, as a result, urticaria appears.
4. Able to provoke mastocytosis, even some drinks or foods. Much depends on how prone a person is to allergic reactions. It is the presence of an allergy to certain components in food that can provoke mastocytosis.
5. Taking medications that harm the immune system or simply do not fit the body, poisoning with toxic substances are the causes of hives. These factors are the most dangerous and intractable, since here it is already possible to judge even the poisoning of the body.

It is generally accepted that it is the release of heparin and histamine from cells that contributes to the narrowing of large vessels and the expansion of smaller ones, which, as a result, leads to disruption of processes in tissues and swelling of some areas of the skin.

Symptoms of mastocytosis

To correctly identify this disease in yourself or loved ones, you need to know its symptoms. As a rule, they consist not only in the defeat of the skin.

The manifestation of mastocytosis on the skin in children and adults is somewhat different. So, mastocytosis in children manifests itself in the form of pink or red spots, which eventually turn into subcutaneous blisters with a clear liquid. After a while, the blisters burst, their contents flow out. In their place, brown spots appear, which can remain on the skin for life.

In adults, everything is not so scary, but the course of the disease can be long. There are small clear nodules or spots that become larger with scratching or rubbing. Over time, they disappear on their own, but then reappear. It can last a lifetime.

Along with skin lesions, there is an increase in body temperature, and in some cases even chills. As a rule, the skin is very itchy, and the spots become bright red. Sometimes the inflammation is so strong that it is even accompanied by an attack of itching, which is impossible to endure.

In especially severe cases in adults, mastocytosis can cause problems with the heart and pressure. Tachycardia usually begins to develop, the pressure is greatly reduced.

Forms of the disease

Mastocidosis also differs in forms, each of which is characterized by its severity. There are 3 main forms of urticaria pigmentosa. But doctors, when making a diagnosis, also specify them. Let us dwell on the main forms of mastocytosis in more detail.

3 main forms of the disease

The first form is skin. Doctors consider it the easiest. As a rule, in most cases it occurs in adolescence. In this case, only the skin is affected, internal organs are not damaged, and no pathologies occur. If treatment is started immediately, then such a pigmented urticaria passes quickly and no longer interferes with the life of an adult.

The second form of this disease is systemic mastocytosis. It develops exclusively in adults. In this case, the skin can both be affected and remain completely unharmed. But it is already much more difficult to cure systemic mastocytosis, since internal organs are damaged.

The third form of mastocytosis is the most terrible. This is mast cell leukemia. It is quite difficult to identify it, since external damage to the skin is not observed. Degeneration of cells occurs, which leads to disruption of the work of all internal organs. It is difficult to treat such leukemia, most often the disease ends in death.

It should also be understood that in the first two forms, if damage to internal organs occurs, then it remains at the same initial level, without progressing further. Sometimes it goes away on its own, but still, treatment should not be neglected.

When conducting tests, the doctor must identify more clearly the form of mastocytosis. There are also forms of this disease at the cellular level, where everything depends on which layer of the skin the unfavorable cell modifications occur.

5 forms at the cellular level

Solitary urticaria pigmentosa most often develops in infants. In this case, a nodule appears on the body, which can reach 5 cm in width. It has a bright color and a fairly dense structure. Usually the disappearance occurs suddenly (without treatment).

The nodular form of mastocytosis can be observed in both adults and children. It is characterized by the presence on the body of many approximately identical dense yellowish nodes. Such nodes, if not treated, merge into plaques. When exposed to damaged areas of the skin, severe pain or itching is felt.

The erythrodermic form of urticaria pigmentosa is considered one of the most unpleasant. The thing is that nodules appear on the body, which are clearly defined, but the shape can be different. They are located close to each other, and when combed, they begin to inflame. As a result, even if a person did not scratch the nodules, sores appear in their place, which are then difficult to eliminate.

Telangiectatic urticaria pigmentosa most often affects women, since changes in cells in most cases are associated with hormonal imbalances. Small brown spots or nodules appear on the chest, arms or legs. Treatment in this case should be to restore normal hormonal levels. Therefore, it makes sense to seek help from a gynecologist.

The manifestations of maculopapular mastocytosis are most similar to those of real urticaria. On some area of ​​the skin, small light yellow or brown vesicles appear, which are clearly limited. Over time, the bubbles fill with fluid, which, after bursting, comes out.

You need to understand that each form of mastocytosis has its own characteristics, so it is very important that the doctor correctly diagnoses. To do this, the patient should pay special attention to the analysis, adhering to the doctor's recommendations before submitting the biomaterial.

Features of the treatment of the disease

It is very important to start the treatment of urticaria pigmentosa on time and correctly.

Such treatment should be prescribed by a doctor, depending on whether the mastocytosis is aggressive or indolent. You can not take any drugs on your own, especially if it is systemic mastocytosis. Therefore, we confine ourselves to considering traditional methods of curing systemic mastocytosis, its other types, and some useful tips.

The doctor must prescribe two groups of drugs to the patient. In this case, one group will be aimed directly at relieving symptoms and normalizing the functioning of organs, and the second will affect the human immune system.

Recently, corticosteroids, cytostatics, and also drugs that have a pronounced anti-inflammatory and anti-allergic effect have become widespread as the main medicines. It is impossible to accurately name the drugs, since they are prescribed individually for each patient. But a common property for all drugs is a decrease in serotonin in the body.

Much attention should be paid to immunity. It is very useful to take vitamin C. It is best to give preference to vitamin complexes.

Some important rules should be observed during the treatment of urticaria pigmentosa. It is necessary to temporarily abandon the long-term use of warm baths, too hot or cold showers. You should be less likely to go outside in extreme heat. Any sudden change in climate can exacerbate the disease.

Much attention should be paid to nutrition. If possible, salty, smoked, fatty or very sweet foods should be avoided. All this can aggravate the situation or even cause an unexpected allergic reaction in the body. You need to focus on fresh vegetables and fruits, natural broths.

If the treatment of urticaria pigmentosa is started on time and correctly, then the prognosis will be favorable. The only exceptions are severe cases of the disease. Therefore, when the first symptoms are detected, seek help from doctors.

Urticaria pigmentosa is a definition for one of the most widespread types of such a disease as mastocytosis, which is systemic, and by its nature is not fully related to urticaria. Mastocytosis is a pathological process that promotes the reproduction and accumulation of mast cells in healthy tissues. Such cells are considered the most important component of human immunity, and on the basis of these data, experts concluded that urticaria, by its nature, is the result of a malfunction of the entire human defense system. People regardless of gender are equally exposed to the disease, while children are at the highest risk.

Mastocytosis or urticaria pigmentosa

Characteristics of the disease

Urticaria pigmentosa, on the basis of statistical indicators, can be classified as a childhood disease, since three-quarters of patients who seek medical help are representatives of this category. Juvenile forms of the disease are characteristic of the pubertal periods of a child's development. The course of this type of disease is accompanied by the formation of pinkish or reddish spots on the skin of the child, which gradually change and become subcutaneous blisters with a watery filling. At the site of these neoplasms, at the end of the healing processes, characteristic traces of a brownish tint remain.

Such spots in children with a corresponding predisposition may disappear completely over time, while for some categories it is possible not only to preserve such a spot, but also to significantly increase it.

In adults, the hallmark of the disease is the process of appearance on the surfaces of the skin of smooth patchy formations with clear boundaries, as well as nodules and vesicle formations, the size of which often does not exceed five millimeters. Such a rash may appear intermittently, since urticaria may have undulating periods of manifestation, when a period of exacerbation is followed by a period of remission and a significant subsidence of symptoms. Mechanical irritation provoked by combing the inflamed areas of the skin, or damage to it, which ultimately leads to the formation of edema and inflammation, can contribute to the exacerbation of the disease. In adults, the onset of the disease can cause the development of its chronic form, which will accompany him all his life.

Urticaria pigmentosa in children may go away on its own, but may remain for life.

Reasons for the development of the disease

To date, there is no established information about what factors are the root cause of the development of mastocytosis, but many factors indicate that the main indicator for determining people at risk is a genetic predisposition. Urticaria pigmentosa is often diagnosed in people who are closely related to those who have previously encountered this problem. Another version is the theory of the infectious type of the occurrence and spread of the disease, since a number of factors indicate such a possibility. Despite this, the vast majority of diagnoses of urticaria pigmentosa are indicated as diseases with unclear etiology.

The symptomatology of mastocytosis develops against the background of mast cell degranulation, which contributes to the release of gaiparin and other hormones, as well as exerting a strong influence on the transformations that take place inside the tissues. These complex active compounds have a significant effect on the increase in the permeability of the walls of the blood vessel. This effect is especially strong on microcirculation, since under its influence the process of narrowing of large and expansion of small vessels occurs. Such processes contribute to the formation of tissue swelling.

Genetic predisposition is one of the causes of mastocytosis

Forms of development of urticaria

Urticaria pigmentosa, the development of which is not associated with problems of the immune system, can be formed under the influence of some physical factors. Among these factors are:

• exposure to the skin of direct sunlight;
• the process of squeezing and rubbing the skin;
• exposing the skin to heat or cold.

In addition, there are exogenous factors that can provoke the development of mastocytosis in children and adults. Among the main reasons capable of such transformations in the body are stressful situations, significant changes in climate and environmental conditions, taking pharmaceuticals, contact with toxic substances, as well as eating certain foods that contain a large number of allergens. In addition to the above classification in adults, as in children, cutaneous mastocytosis can manifest itself in several main forms:

• systemic;
• malignant;
• skin.

The form of systemic mastocytosis is typical for older and middle-aged people. It manifests itself by a negative impact on the tissue of internal organs or several of them. For this form of the disease, the presence of an external manifestation is not necessary. Malignant forms of mastocytosis are called mast cell leukemia, which is characterized by malignant degeneration of the mast cell of the body. The course of the disease is often not accompanied by the formation of a rash or other signs of skin lesions. The main negative impact is taken by the organs of internal systems. The complexity of the disease lies in the fact that it is often detected in the late stages of the development of the disease, which are poorly amenable to therapy, and can cause death. Cutaneous forms of mastocytosis can manifest in both adult and infant form.

1. The infantile form of mastocytosis often affects children's organisms, and disappears only with the onset of puberty. For this form of the disease, it is not typical to have a negative effect on the internal organs, but there is an active appearance of neoplasms and irritations on the skin. A child who has experienced a disease at an early age may face the problem of the transition of the disease to the adult stage.
2. For adult and adolescent mastocytosis, inflammation and irritation of the skin is not the only negative effect, since the disease affects internal organs such as the kidneys, heart, and also the organs of the digestive system. The progression of such complications in most cases is not observed, and the disease itself passes from the adult skin form into a systemic disease.

Infantile mastocytosis may resolve with the onset of puberty

Symptoms of urticaria pigmentosa

Most of those patients who are faced with the problem of developing urticaria pigmentosa have complaints exclusively of the external manifestations of the disease, while this disease may have other disturbing symptoms. So, among the most common hidden symptoms of the disease, one can single out a decrease in blood pressure, which occurs for no apparent reason. Sharp and unreasonable jumps in temperature, paroxysmal tachycardia, as well as severe itching, formed against the background of reddening of the skin. The main danger of these symptoms is that their presence indicates the presence of malfunctions in one of the organs, or some systems. Based on this, timely seeking qualified medical help is a prerequisite when the first symptoms are detected, since only the right treatment can give a result. Depending on the impact on certain layers of the epidermis, the form of the skin manifestation of urticaria pigmentosa may change. To date, experts have identified 6 main types, which differ in the form of manifestation.

• The nodular form is manifested by the formation of a large number of nodular neoplasms, which may have a smooth or bumpy structure. Such growths may have a yellow, reddish or pink color, which differ significantly from healthy areas of the skin. In some cases, the proximity of several nodules can provoke their fusion into a large plaque. It is not possible to cause pathological reactions by mechanical action on such neoplasms.
• The maculopapular form can manifest itself by the formation of a large number of dark spots on the skin, or small nodular neoplasms. Here there is a clear limitation of lesions. Mechanical impact on such areas provokes the formation of small bubbles, which in appearance and structure resemble rashes provoked by real urticaria.
• The solitary form, which is characteristic of infants, can cause the formation of nodules, up to five centimeters in size, which to the touch, in their structure, resemble rubber or rubber products.
• In the erythrodermic form, the affected areas of the skin acquire a yellowish-brown color, are significantly thickened, and their edges receive clear, outlined boundaries. Characteristic of this form is the presence of severe itching, which provokes the patient to mechanical action and combing the inflamed areas. The result of such manipulations is the appearance of cracks and sores on the skin exposed to the disease.
• The bullous form of urticaria manifests itself through the formation of numerous bubbles, the growth of which can be provoked by even the smallest impact, which similarly affects the presence and strength of the sensation of itching. Transitions of this form into systemic types are recorded quite rarely, although the lack of adequate and timely treatment can lead to a continuous lesion of the skin, which will lead to redness, peeling, and an increase in resistance to therapeutic manipulations. All this will greatly complicate the treatment.
• The telangiectatic form of urticaria causes affected areas of the skin to turn red-brown. The localization zone of such spots in most cases is located in the chest area, as well as in the area of ​​\u200b\u200bthe arms or legs. In children, this disease is diagnosed quite rarely, since the risk group is mainly formed by middle-aged female representatives.

Telangiectatic mastocytosis mostly affects older adults.

Diagnosis of urticaria pigmentosa

In children and middle-aged people, the presence of this type of disease can be determined already as a result of primary examinations, as well as anamnesis. In order to confirm the established diagnosis, as well as to determine the nature of the disease and methods of its therapy, a biopsy of skin areas should be performed. Given the fact that each of the above types of disease has its own characteristics, their treatment also differs in some key nuances. To establish a complete clinical picture, the doctor who treats the patient may prescribe a series of tests that, by providing a complete picture, will help increase the effectiveness of treatment, as well as avoid complications and the development of concomitant diseases.

In addition, some specialists, in order to ensure timely treatment of systems and organs that may be affected by urticaria, use ultrasound types of research, as well as computerized tomography of bone tissues.

Ways to treat urticaria pigmentosa

The treatment that urticaria pigmentosa and mastocytosis should undergo must necessarily have an integrated approach that ensures the parallel elimination of external and internal factors. To provide external therapy, in most cases, creams, ointments and rubbing are used that can reduce itching, as well as eliminate inflammation. Internal drugs work to reduce the body's production of serotonin. Medicines prescribed for this disease must also fight allergens. Cutaneous mastocytosis is also treated with corticosteroids and cytostatics.

In order for the treatment to be effective and not entail negative consequences for the body, it must be carried out under the close attention of physicians, who must adequately respond to the clinical picture and, if necessary, make changes to the forms and methods of treatment.