What is mastocytosis (urticaria pigmentosa) and what are the causes of the appearance in a child? Treatment and photo of children with a rash. What is urticaria pigmentosa, its symptoms and how to treat it Urticaria pigmentosa treatment

Urticaria pigmentosa (mastocytosis) is one of the rare and little-studied diseases. There is no reliable information about the prevalence of this disease, its etiology and pathogenesis. There are the following forms of mastocytosis:

1. pigment spotted mastocytosis
2. knotted
3. bullous
4. papular
5. mastocytoma
6. diffuse

1. insulated non-aggressive:
   a. asymptomatic
   b. skin
   in. systemic with visceral manifestations
2. mastocytosis with hematological disorders:
   a. myeloproliferative
   b. myelodysplastic
3. aggressive
4. mast cell leukemia

The disease usually appears during early childhood and often disappears spontaneously after a few years at puberty. The course of the disease is usually benign. But, approximately, in 30% of patients, hyperemia, itching, urticaria elements periodically appear, in less than 15% vomiting, deep fainting, shock occurs. These manifestations are probably associated with the release of histamine by mast cells and are often combined with increased urinary excretion of free histamine and its metabolites.

Systemic mastocytosis, in which mast cells diffusely infiltrate the liver, spleen, gastrointestinal tract, and bones, is very rare. Sometimes mast cell leukemia develops.

We observed a patient with a combination of urticaria pigmentosa and anaphylactic shock to novocaine. We present our observation. Patient Z., 68 years old, 12.02.1999 was transferred to the allergological department of the Vitebsk Regional Clinical Hospital from the intensive care unit of the 3rd city hospital, where she was due to anaphylactic shock due to the introduction of novocaine. From 18.01.1999 to 8.02.1999 the patient was treated in the proctology department of the 2nd city hospital, where she underwent a polypectomy under local anesthesia with lidocaine. Before discharge, novocaine was introduced into the anus. After 15 minutes, the patient felt weak and lost consciousness. Assistance was provided in the intensive care unit of the 3rd city hospital.

Anamnesis of life. Of the past diseases, he notes an appendectomy in 1984. For about 10 years, arterial pressure periodically rises. Takes clonidine. Pain in the heart does not note. There are two children (physiological childbirth).

Allergy history. In 1984, after an appendectomy in a hospital, while taking some medicine in a tablet (sulfanilamide?), a sharp weakness developed. There were no reactions to food products. There are no relatives of patients with allergic diseases.

Objectively. The condition is satisfactory. On the skin of the back, chest, abdomen, extremities - a pigmented rash against the background of telangiectasias. Any finger pressure on the skin led to the formation of erythema (the Darier-Unna phenomenon).

Visible pink mucous membranes without pathological elements. Lymph nodes accessible for palpation are not enlarged. The isthmus of the thyroid gland is palpable. The mammary glands are soft, painless. Vesicular breathing in the lungs. Heart sounds are muffled, rhythmic, heart rate is 60 per minute. The abdomen is soft and painless. The liver and spleen are not enlarged. Stool and diuresis are normal.

Complete blood count (15.02.99). erythrocytes -4.0 x1012 / l; hemoglobin - 125 g/l; leukocytes - 7.2x109 / l; p - 1%, s - 60%, e - 9%, l - 29%, m - 1%; ESR - 3 mm/h.

Biochemical blood test (12.02.99). Urea - 8.7 mmol/l; ALT - 0.14 µmol/h H l; AST - 0.23 µmol/h H l; K - 4.08 mmol / l; Na - 141.2 mmol/l; CL - 102 mmol/l; total protein - 85 g/l.

Urinalysis (11.02.99). Specific gravity - 1017, color - light yellow, acidic reaction, complete transparency, protein - no, sugar - no; microscopy: epithelium 1-2 in the field of view, leukocytes 1-2 in the field of view, salts - uric acid crystals. No worm eggs were found in the feces (17.02.99).

ECG (18.01.99). Sinus rhythm 85 per minute. Horizontal EOS. Single ventricular extrasystoles. ECG (11.02.99). Sinus rhythm 60 per minute. Horizontal EOS. Left ventricular hypertrophy.

Examination by an ophthalmologist (16.02.99). VOD - 0.9; VOS - 0.7. The fundus of the eye - the discs are pale pink, the boundaries are clear; retinal vessels of normal size. Examination by a neurologist (18.02.99). Neurological pathology was not revealed. It was impossible to conduct a morphological study of the skin biopsy, since the patient had an anaphylactic reaction to novocaine.

Given the long history of high blood pressure, the absence of changes in the fundus, as well as periodic heart rhythm disturbances (see ECG dated January 18, 1999), without subjective sensations, one can think of a paroxysmal release of histamine by mast cells and a reflex effect on the activation of the adrenal medulla with the release catecholamines. Attention is drawn to the combination of urticaria pigmentosa and anaphylactic shock, which can lead to a condition that is difficult to treat.

Urticaria pigmentosa is a skin disease that leads to a violation of pigmentation. The second name of the disease is mastocytosis. In most cases, it is diagnosed in children, but it can affect adults.

The reasons

Urticaria is a disease that affects the epidermis, under the influence of certain factors. The disease is characterized by the presence of rashes of red or pink color, severe itching.

Urticaria pigmentosa does not fully belong to this group of diseases. Mastocytosis is considered a pathological condition of the body, in which there is a rapid multiplication of mast cells in healthy tissues. During their destruction, a large amount of histamine is released, which provokes a skin reaction.

Urticaria pigmentosa was discovered and described in 1869. It is included in the International Classification of Diseases and has the ICD-10 code - Q82.2 - mastocytosis.

The exact cause of the disease has not been established. There are some factors that can provoke the development of such urticaria. It can be of immune and non-immune nature of origin.

Factors:

Many scientists are inclined to believe that the main cause is a hereditary factor, as well as inflammatory processes that develop as a result of infectious diseases. To provoke the development of urticaria can be a reaction to the MMR vaccination.

The shade of the skin, characteristic of the pigment type of the disease, occurs as a result of an increase in the number of melanocytes and, as a result, melanin in the epidermis layer.

Forms and symptoms of urticaria pigmentosa

Urticaria pigmentosa has several different forms.

Classification:

• Systemic. This type of urticaria is often diagnosed in adults. It is characterized by damage to internal organs, external signs of the disease may be absent.
• Malignant. The severe form, in which mast cell malignancy occurs, is dangerous to humans. Epidermal lesions are often absent, internal organs suffer. Urticaria is characterized by a severe course, it is practically not amenable to treatment. Causes death.
• The skin form of pigmentary disease does not extend to the internal organs. Diagnosed in infants, disappears by adolescence. Sometimes it can be diagnosed in adults.
• In adolescents and adults, the adverse effect of urticaria extends to the internal organs, but the disease does not develop quickly and is not systemic.

In addition to this division, single and generalized (multiple) skin rashes are distinguished. The second type of pigmented urticaria is more common.

Symptoms

In most cases, patients pay attention to the external signs of the disease, not noticing other changes in the body. There are several types of changes in the skin with pigmented urticaria.

Kinds:

• Maculopapular. Diagnosed regardless of age, characterized by spots with a clear border.
• Erythrodermic. The spots have a yellowish color, uneven edges. There is intense itching. Diagnoses at any age, children do not form blisters.
• bullous. On age spots, the formation of bubbles in multiple quantities is noted. It most often affects young children.
• Nodal. Small nodules, single or multiple, form on the epidermis. Affect newborns or children under two years of age.
• diffuse. The epidermis becomes thicker, a yellowish tint appears, deepening of the skin folds is noted. Develops regardless of age.
• Telangiectatic. Red-brown spots form on the epidermis. It is more commonly diagnosed in adults, especially women.

Cutaneous mastocytosis in children and adults

Urticaria pigmentosa in children is a benign form of the disease. On the skin, the formation of pink or red spots is noted, intense itching appears.

After a while, the spots turn into blisters filled with liquid contents. When rubbing or puncturing the pigment spot, there is a sharp reddening (Daria-Unna phenomenon) and increased itching. Exacerbations are observed when taking warm baths, prolonged exposure to the sun, mechanical pressure.

Often lesions are diagnosed on the body, arms, legs. Sometimes the appearance of not bubbles, but nodules of red color is noted.

In children, the skin becomes thicker and rougher, acquires a yellowish tint, the folds appear more pronounced.

Sometimes children develop a solitary mastocytoma. A tumor forms on the body, reaching a size of five centimeters. Basically, this is a single formation. When the tumor is damaged, bubbles form on it.

Unpleasant symptoms of urticaria pigmentosa disappear by puberty.

Mastocytosis in adults

In adults, urticaria pigmentosa develops similarly. First of all, red spots appear on the entire surface of the body. After some time, the spots may darken, change their shape.

The development of urticaria has a periodic course. The symptoms disappear, then reappear. With a systemic appearance, damage to internal organs gradually occurs, which can cause death. Patients often do not notice other signs of the disease.

What to look for:

• Constant drop in blood pressure
• Feelings of weakness and apathy
• Painful sensations in the muscles
• Anaphylactic shock,
• Violation of the respiratory system.

Such symptoms should alert a person and are a reason to visit a medical facility.

Diagnosis and treatment

Before prescribing treatment for the disease, various examinations are carried out. High-quality diagnostics allows you to make an accurate diagnosis and select drugs that can cope with pigmentary disease.

External signs of pigmented urticaria are similar to the symptoms of other skin diseases. Therefore, it is necessary to differentiate mastocytosis from them.

Research:

• Blood and urine tests,
• skin biopsy,
• histamine tests,
• Ultrasound procedure,
• X-ray,
• Magnetic resonance imaging,
• CT scan.

Once the diagnosis is made, the appropriate treatment is selected.

Therapy is prescribed individually for each patient and includes the use of various means.

In mild forms in children, treatment is not prescribed due to the possible disappearance of all signs of pigmented urticaria in adolescence.

Treatment:

• Antihistamines are prescribed - Diazolin, Zirtek, Tavegil. Render anti-allergic effect, reduce itching and discomfort.
• Antiserotonin agents are used - Ketotifen, Bikarfen. Drugs inhibit the production of histamine in the body.
• To eliminate external signs, corticosteroid drugs are used - ointments, creams, gels.
• Medications based on cromoglyceric acid - Nalkrom, Kromoheksal - have an antiallergic effect.
• With a nodular form, Histaglobulin is prescribed.

In some cases, physiotherapy procedures are prescribed - ultraviolet irradiation.

In the absence of results from conservative treatment, surgical intervention is used.

At all stages of therapy, the patient adheres to proper nutrition. All fried, smoked, pickled dishes, sweets, carbonated drinks, coffee, alcoholic drinks are removed from the diet. Preference is given to fermented milk products, fruits, vegetables, green tea.

Folk remedies

In the absence of contraindications, it is allowed to use folk remedies for the treatment of the pigmented form.

Funds:

• The beets are cleaned, cut into small pieces, poured with three liters of water. Leave in a dark place for six days. Ready infusion is consumed in a large spoon three times a day and used to make compresses on inflamed areas.
• Drink half a glass of celery juice three times a day.
• An equal amount of sage, chamomile, calendula, celandine, valerian is mixed and used to make an infusion. The finished product is used for therapeutic baths.

Folk remedies are an addition to the main treatment.

Forecast and who to contact

What is the danger of the disease? Urticaria pigmentosa in its neglected form and in the absence of high-quality treatment can cause death of a person.

Subject to all doctor's prescriptions, the prognosis is usually positive. In children, the disease often resolves on its own by adolescence.

In adults, urticaria pigmentosa can progress, but long-term remissions are not excluded.

Which doctor to contact

The treatment of the pigmented form of urticaria is carried out by a hematologist, therapist, oncologist, dermatologist. It is necessary to visit all specialists to exclude malignant diseases and adhere to appointments.

Urticaria pigmentosa can cause a lot of discomfort to the patient. When the first signs of the disease appear, it is necessary to visit a medical institution and adhere to the prescribed treatment.

Doctor's webinar about this urticaria - video

There are two forms of mastocytosis: cutaneous and systemic. The cutaneous form is subdivided into generalized cutaneous (urticaria pigmentosa, persistent macular telangiectasia, diffuse mastocytosis) and mastocytoma (tumor, usually solitary).

Urticaria pigmentosa is the most common form of skin mastocytosis. At the beginning of the disease, often in children, there are itchy pink-red spots, which eventually transform into blisters. After themselves, the blisters leave persistent brownish-brown spots. In adults, the disease begins with the formation of hyperpigmented macules or papules. Spots and papules are flat, up to 0.5 cm in diameter, have a rounded shape, sharp borders and a smooth surface without signs of peeling. They are located on the skin of the body, are not numerous, have a light gray or pink-brown color. Over time, papules spread to other areas of the skin (upper and lower limbs, face), have a spherical shape, dark brown or dark brown in color, sometimes with a pink-red tint. Often, the process stops, persisting for many years, in the future it can progress with the development of erythroderma, affect internal organs, which ends in death.

Urticaria pigmentosa in children is benign. The disease begins with the appearance of itchy urticarial rashes, which after a few years are transformed into papular elements. At the onset of the disease, blisters (vesicles) may appear on apparently healthy skin or on spots and papules, characterized by swelling and a distinct pink-red tint. Clinically, urticaria pigmentosa in children is distinguished by a clear exudative component. Sometimes elements disappear without a trace. The inflammation phenomenon, or the Darier-Unna phenomenon, is important: when rubbed with fingers, or with a spatula or with a needle prick, the elements become swollen, pink-red color and itching of the skin increase. Exacerbation is noted after friction, pressure, thermal procedures (hot baths, insolation).

The skin form can manifest itself as maculopapular, bullous rashes, diffuse, including erythrodermic, telangiectatic changes, and also quite rarely in the form of an isolated focus - mastocytoma. The most common are small-spotted and papular rashes, spreading mainly in childhood. They are located mainly on the trunk, somewhat less often on the limbs, rarely on the face, have rounded or oval outlines, reddish-brown color. After friction, the elements acquire an urticarioid-like character. At the same time, there may be nodular formations, usually not numerous, with more intense pigmentation. Expulsions can merge, forming plaque and diffuse lesions, in rare cases acquire a pachydermic character with a pronounced diffuse infiltration of the skin.

The persistent spotty telangiectotic form occurs in adults, looks like freckles, small telangiectasias are visible against a pigmented background.

Diffuse mastocytosis is characterized by thickening of the skin, which has a doughy texture, a yellowish tint and resembles a picture of pseudoxanthoma. Deepening of skin folds is noted. Foci are often localized in the axillary cavities, inguinal folds. Cracks and ulcers may appear on the surface of the lesions.

Cystic and atrophic forms are also described: blisters are tense, with transparent or hemorrhagic contents, acantholytic cells are absent in them, Nikolsky's symptom is negative.

Mastocytosis nodosa occurs predominantly in newborns and children in the first 21 years of life.

Clinically, there are three types of nodular mastocytosis: xanthelasmoid, multinodular, and nodular-confluent.

The xanthelasmoid variety is characterized by isolated or grouped flat nodules, or nodular elements up to 1.5 cm in diameter, oval, with sharp boundaries. The elements have a dense texture, a smooth or orange-peel surface and a light yellow or yellowish-brown color, which makes them similar to xanthelasmas and xanthomas.

With multinodular mastocytosis, multiple hemispherical dense nodular elements with a smooth surface are scattered throughout the skin, which have a diameter of 0.5-1.0 cm, pink, red or yellowish.

The knotty-confluent variety is formed as a result of the merging of knotty elements into large conglomerates located in large folds.

With nodular mastocytosis, regardless of its varieties, the Darier-Unna phenomenon is weakly expressed or not detected. In most patients, urticarial dermographism is determined. A characteristic subjective symptom is itching of the skin.

With systemic mastocytosis, damage to the internal organs is noted. The disease manifests itself in the form of mast cell leukemia (a malignant form of mastocytosis).

The erythrodermic form, which is also rare, in adults, unlike children, proceeds without vesicular reactions.

Diffuse-infiltrative, gelangisctatic and erythrodermic forms of mastocytosis are considered potentially systemic diseases.

Bullous mastocytosis develops in children in infancy or early childhood. Bubbles can be on the surface of maculopapular rashes or plaque lesions, sometimes they are the only skin manifestation of the disease (congenital bullous mastocytosis), which is considered less favorable prognostically.

Solitary mastocytoma has the appearance of a small tumor-like formation or several closely spaced nodules, on the surface of which blistering is characteristic in children. The prognosis for this variant of urticaria is the most favorable. In most cases, urticaria pigmentosa, which developed in childhood, regresses on its own by puberty. On average, systemic lesions are observed in 10% of patients, mainly in the case of the development of mastocytosis in adults.

Urticaria pigmentosa is commonly referred to as one of the most common forms of mastocytosis, a systemic disease that, in fact, is not urticaria.

Mastocytosis is the process of pathological reproduction and accumulation of mast cells in living tissues. Mast cells are a significant part of the body's immune system, so there is reason to believe that urticaria pigmentosa results from disorders in the immune system.

On average, this disease affects both sexes in approximately equal proportions.

The vast majority of cases, about three out of four cases, occur in childhood (juvenile form) and disappear at the beginning of puberty. Pinkish or reddish spots appear on the body of children, gradually changing into subcutaneous blisters with a watery filling, which, after healing, leave brownish marks. In some patients, these traces may disappear completely after some time, in others, the spots remain and even increase in size.

In adult patients, the disease begins with the appearance on the skin of smooth, well-defined spots, vesicles or nodules no larger than 5 mm in size. These rashes are periodic (exacerbations are interspersed with periods of improvement), with mechanical irritation (rubbing) or damage, they can swell and even become inflamed. Urticaria pigmentosa that occurs in adults can accompany a person throughout his life.

Causes of the disease

Regarding the causes of the development of mastocytosis, there is no generally accepted theory. There are certain arguments both in favor of a genetic predisposition (frequent cases of pathology among people who are fairly closely related), and in favor of the infectious theory. A large proportion of cases are classified as urticaria pigmentosa of unknown etiology.

Symptoms of mastocytosis develop as a result of degranulation of mast cells, as a result of which histamine and heparin are released, which have a strong effect on the processes occurring in the tissues. Under the influence of these complex and active compounds, the permeability of the walls of blood vessels increases significantly, especially in the microcirculation system, large vessels narrow, small ones, on the contrary, expand, which generally leads to tissue edema.

Urticaria pigmentosa can be both immune and non-immune in nature.

Non-immune forms develop under the influence of the following physical factors:

• direct sunlight;
• squeezing;
• friction;
• warm;
• cold.

Among the exogenous factors of non-physical properties are the following reasons:

• stress;
• change in climatic conditions;
• pharmaceutical preparations;
• toxic substances;
• some food.

Forms of the disease

The following classification of mastocytosis is generally accepted:

• systemic form (develops mainly in adults), which is characterized by damage to the tissues of internal organs, and skin lesions may be present or absent;
• malignant (otherwise mast cell leukemia), characterized by malignant degeneration of mast cells. Occurs, as a rule, without skin lesions, but leads to infiltration of internal organs, is difficult to treat and in most cases leads to an early death;
• cutaneous form, which includes adult and infantile mastocytosis. The latter occurs at an early age and disappears at the onset of puberty. With this form, skin phenomena are usually not accompanied by lesions of the internal organs. Occasionally there are cases of transition to the adult form;
• in adult and adolescent mastocytosis, in addition to skin phenomena, damage to internal organs occurs - the kidneys, heart, and some organs of the gastrointestinal tract. However, these lesions usually do not progress, although cases of transition of cutaneous adult mastocytosis to the systemic form are known.

Symptoms of the disease

Approximately half of patients who are diagnosed with urticaria pigmentosa go to the doctor only with complaints about the condition of the skin. However, sometimes the following symptoms are observed:

• drop in blood pressure for unknown reasons;
• seemingly unreasonable increase in body temperature;
• attacks of paroxysmal tachycardia;
• bouts of itching with simultaneous reddening of the skin.

Such signs indicate damage to internal organs.

Forms of mastocytosis

Forms of skin manifestations depend on which layers of the epidermis accumulate active substances secreted from mast cells.

Experts identify five types of skin changes in this disease.

Nodular - characterized by the appearance of many convex seals or nodules, smooth or bumpy. Usually the color of these formations differs from the color of healthy skin: yellowish, red, pink. Sometimes the nodules are so dense that they merge into plaques. Mechanical action does not cause a pathological reaction.

Maculopapular - manifests itself in the form of the formation of many dark-colored spots or small nodules on the skin. Lesions on the skin are quite clearly limited. With mechanical action, small bubbles are formed, very reminiscent of the symptoms of real urticaria.

Solitary (infant form) - characterized by the formation of a node up to 50 mm in size, similar to the touch with rubber. Such nodes are called mastocytomas, they can have a smooth or wrinkled surface, are located on the trunk, arms or neck. Most often, only one macystoma occurs, less often - up to four. These formations are characterized by their sudden disappearance.

Erythrodermic - characterized by the formation of yellowish-brown lesions on the skin, dense to the touch, with jagged edges and clearly defined boundaries. This form of the disease is characterized by rather severe itching, which gradually leads to scratching, and as a result - to cracks or sores.

Even with relatively light exposure, increased itching and blistering may occur. Perhaps the development of the so-called bullous form with multiple vesicles. The transition to the systemic form is relatively rare, but it is possible to develop a continuous skin lesion with redness, peeling and resistance to treatment.

The telangiectatic type is characterized by the appearance of red-brown spots on the affected skin, located mainly on the chest, legs or arms. It develops most often in adult women.

Diagnosis of the disease

Urticaria pigmentosa is diagnosed by initial examination and questioning, but a skin biopsy is usually performed to confirm the diagnosis.

Each of the types of development of pathology is characterized by different types of distribution of infiltrates in the epidermis.

In the solitary form, infiltrates even extend to the subcutaneous tissue, and in the erythrodermic form, they accumulate mainly in the surface layers.

In order to clarify the diagnosis, blood and urine tests are prescribed.

An ultrasound examination of the internal organs may be prescribed, and in some cases, computed tomography of the bones, since with mastocytosis, damage to the internal organs and bone tissue is possible.

There are a number of skin formations and various diseases that have similar external manifestations or give similar test results. Therefore, the diagnosis of mastocytosis is usually complex.

Treatment

After an accurate diagnosis, urticaria pigmentosa requires complex treatment under the guidance of a specialist. Used drugs that reduce the production of serotonin, anti-inflammatory, corticosteroids, cytostatics and antiallergic drugs. Sometimes surgery may be required to remove the mast cell.

The disease is often intractable, sometimes it is necessary to confine itself to symptomatic treatment, immunomodulatory therapy and preventive measures.

Please note: people diagnosed with urticaria pigmentosa should be careful about any thermal procedures, including sunbathing and sauna visits, as they can provoke an exacerbation of the disease.

Forecast

The infantile form, with the right treatment tactics, may well be cured and in most cases disappear completely by adolescence. Those who become ill in adolescence have a lower chance of a full recovery, as for adult patients, their disease, as a rule, tends to progress, although there are also long-term periods of remission.

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 the 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 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.