home Audience 12 What is urticaria pigmentosa and how to fight it? What is urticaria pigmentosa, its symptoms and how to treat it Treatment with traditional methods

What is urticaria pigmentosa and how to fight it? What is urticaria pigmentosa, its symptoms and how to treat it Treatment with traditional methods

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Mastocytosis is a disease that is caused by the pathological proliferation and accumulation of mast cells, which are more often called mast cells, in tissues and organs. One of the varieties of the disease, manifested by the formation of numerous rashes on the human body, is called “urticaria pigmentosa.”

The disease is considered rare: according to medical statistics Out of every thousand cases of adult patients visiting a dermatologist, only one is diagnosed with mastocytosis. In children, urticaria pigmentosa occurs twice as often, and most patients are children in the first two years of life.

The first time a condition in which hyperpigmented, itchy spots appear on human skin was recorded in 1869 and was called “urticaria pigmentosa.” However, ten years later, the discovery of special mast cell cells by the German immunologist Paul Ehrlich showed that urticaria new disease has a very distant relationship. After the other German doctor Paul Gerson Unna proved in 1889 that it is mast cells that cause the external symptoms of the disease, which became known as “mastocytosis.”

By modern classification According to the World Health Organization, there are two forms of mastocytosis: cutaneous, still called urticaria pigmentosa, and systemic, of which mast cell leukemia is one type.

The first type of mastocytosis occurs in most cases in very young children and is characterized by external symptoms, practically without affecting internal organs.

Systemic mastocytosis is more common in adults, is sluggish in nature with periodic relapses and can last until the end of life. The disease may not have skin manifestations, but affects the liver, spleen, gastrointestinal tract and bone marrow. The most unfavorable prognosis is for the malignant form of the disease, which progresses rapidly and often leads to the death of the patient.

Causes of the disease

Despite the fact that mastocytosis has been known for almost a century and a half, the exact cause of its occurrence and the mechanism of development have not yet been identified. According to some geneticists, the disease is hereditary and caused by a specific chromosome pathology. However, this version has not received official recognition, since studies have shown that only half of the patients in the family had cases of mastocytosis.

There is an opinion that the disease is associated with disturbances in the process of hematopoiesis and mastocytosis refers to malignant pathologies lymphoid and hematopoietic tissues.

However, it is reliably known that the appearance of symptoms of mastocytosis is caused by proliferation (from Latin words“proles” - offspring and “fero” - to carry), that is, the proliferation of mast cells and their increased release of allergy mediators: histamine, heparin, serotonin, cytokines.

In this case, various factors can trigger the proliferation mechanism. The most common ones include the following:

prolonged exposure to cold or heat;
insect bites, snake bites, jellyfish burns;
uncontrolled use for a long time medicines, including acetylsalicylic acid, B vitamins, local anesthetics, corticosteroids;
consumption of certain foods (spices, coffee, marinades and others);
alcohol and drug abuse.

Mastocytosis can be triggered by nervous overload, stress, climate change, hormonal changes. Newborn children can develop urticaria pigmentosa if one of the listed factors occurred in a woman during pregnancy.

According to doctors, the peculiar coloration of the skin, which led to the name of the disease “urticaria pigmentosa,” is caused by accumulation in the subcutaneous layer of large quantity melanin, which appears when melanocytes interact with mast cells.

Symptoms of mastocytosis

In 75% of cases, the dermatological form of mastocytosis is diagnosed in children from birth to 2 years. Urticaria pigmentosa is very rare in older children, adolescents and adults. No significant differences by gender were recorded.

Classification of mastocytosis

The disease appears suddenly, and the severity of the main symptoms depends on the patient’s age, the form of the disease, the state of the immune system and other factors.

By appearance skin rashes There are the following types of cutaneous mastocytosis:

maculopapular. Formation of a whole scattering of small hyperpigmented spots on the body brick color. When you press on them or slightly rub, voluminous blisters appear, reminiscent of the rashes characteristic of real urticaria (urticaria). In dermatology, this phenomenon is called “Darier-Unna syndrome”;
knotted. Many nodules of pinkish, scarlet or ocher color with a diameter of about 10 mm appear on the human body. They are smooth or with small tubercles and sometimes grow together into a large formation. When touched or pressed, the nodes do not change;
solitary. Typically appears in infants and toddlers under 12 months of age. It is characterized by the appearance of one (sometimes several) orange or dark yellow mastocytes - nodes up to 40-60 mm in diameter. Their surface is similar to the peel of an orange, and their tactile sensations resemble rubber. Located mainly on the neck and upper torso. The Daria-Unna test gives positive result: when pressure is applied to the skin, blisters filled with liquid appear;

erythrodermic or diffuse occurs at any age. Light brown spots appear under the armpits, in the intergluteal space and other large folds irregular shape with clear boundaries and a very hard surface on which cracks often form. Even a slight touch to these areas leads to large blisters. One of the symptoms is severe itching, which is why abrasions, scratches and scratches often appear on the body. In the generalized form with big amount papules can provoke histamine shock and death;
telangiectatic. Usually occurs in women between 25 and 50 years old. It is expressed in the appearance of large red brick spots of irregular shape on the chest and limbs. The Daria-Unna symptom is pronounced, the patient is also bothered by unbearable itching and burning when touched. In severe cases, it can cause damage bone tissue;
bullous The formation on the skin of children of many small blisters filled with liquid, which appear even with slight pressure. Often accompanied by redness and peeling of the skin over large areas of the body.

In addition, according to age, urticaria pigmentosa is classified as follows:

Based on the number of formations that appear on the skin, the following forms of urticaria pigmentosa are distinguished:

localized – one or more mastocytes are formed;
generalized – the rash covers most bodies.

The second form of the disease is diagnosed much more often.

Symptoms of urticaria pigmentosa

With the dermatological form of mastocytosis, patients develop the following symptoms:

skin redness;
the appearance of spots or nodes of various sizes depending on the type of disease;
burning and pain when touched;
formation of fluid-filled blisters on the body when pressed or rubbed;
increased body temperature;
decreased blood pressure;
heart failure.

Sometimes the disease is accompanied by headaches, muscle pain and work disturbances. digestive system: nausea, vomiting, flatulence, diarrhea. A sick child becomes irritable and capricious, often crying. Excruciating itching causes loss of appetite, insomnia, and nervous breakdown.

IN severe cases, with a large release of histamine, histamine shock can develop. Its symptoms are:

a sharp decrease in blood pressure;
decreased muscle tone;
fast or weak pulse;
decrease in body temperature;
loss of consciousness.

This condition requires immediate medical care, as it can cause cardiac arrest and death.

Symptoms of systemic mastocytosis

The systemic form of the disease is characterized by the penetration of a large number of mast cells not only into the subcutaneous fat layer, but into the bone marrow and many internal organs.

The main signs of systemic mastocytosis are an increase in the size of the organ “attacked” by mast cells and disruption of it normal functioning. In addition, patients are often bothered by the following symptoms:

Sometimes blisters and spots may appear on the body, as in dermatological urticaria. They can be isolated or generalized, disappear or appear over time.

The disease can last for years with periodic relapses. Over time, the sick person develops osteoporosis (softening of bone tissue) or osteosclerosis (replacement of bone tissue with connective tissue). The most severe complication is hemorrhagic syndrome leading to death.

At severe defeat bone marrow and the penetration of large numbers of mast cells into the circulatory system, mast cell leukemia can occur. As a result, the patient develops multiple organ failure, which leads to death.

Treatment of the disease

When areas with strong pigmentation and rashes appear on the body, accompanied by one or more additional symptoms, you need to make an appointment with a dermatologist at the clinic, medical Center or a skin and venereal disease clinic. Only experienced doctor will be able to establish an accurate diagnosis and prescribe the necessary treatment.

Diagnosis of the disease

To the complex diagnostic measures includes a visual examination of the affected areas and a survey of the patient to take a medical history and identify the causes of the disease. To do this, the doctor requires the following information:

the presence of dermatological and other diseases in parents or close relatives;
living and working conditions;
diet (for infants - from the mother during pregnancy);
list of medications taken;
bad habits.

To identify chronic diseases The following laboratory tests are prescribed:

blood tests: general, biochemical, tumor markers;
general urine analysis;
computed tomography of bone tissue;
ultrasound examination of the abdominal organs;
radiography of internal organs.

The results obtained will allow us to differentiate urticaria pigmentosa from the following diseases with similar symptoms:

lymphogranulomatosis;
hemangioma;
pemphigus of newborns;
pigmented nevus;
dermatofibroma.

If necessary, carried out histological examination biomaterial taken from the affected area and bone marrow biopsy.

Based on the measures taken, the doctor makes a diagnosis and prescribes treatment.

Treatment of mastocytosis

If urticaria pigmentosa in young children does not manifest itself as multiple formations on the body and signs of systemic lesions, then therapy is not prescribed. In most patients, self-healing occurs during puberty and the signs of the disease disappear. At the same time, the child must be constantly under the supervision of a pediatric dermatologist or pediatrician in order to notice a possible deterioration of the condition in time.

Since the etiology of the disease and the mechanism of its development are not well understood, there is no uniform treatment method. Therapy is most often used, depending on the type of mastocytosis, the main symptoms and the individual characteristics of the person.

The main thing in therapy is to block the process of proliferation of mast cells and reduce the level of histamine and other substances that provoke the development of symptoms of the disease.

For this purpose, the following groups of medications are prescribed:

antihistamines: Allergostop, Fenkarol, Cetrin, Claritin, Gismanal, Erius, Zirtec;
antiserotonins: Ketanserin, Lisuride, Ketotifen, Bicarfen;
anti-bradykinin: Anginin, Prodectin, Parmedin;
hormonal: Prednisolone, Dexamethasone;
containing cromoglyceric acid: Nalkrom, Intal.

External antipruritic ointments are applied to the affected areas:

non-hormonal: Fenistil gel, Psilo balm, Skin Cap, Soventol, Bepanten, Panthenol;
hormonal: Triderm, Akriderm, Advantan, Beloderm.

In case of systemic form of mastocytosis, the use of cytostatics is indicated to prevent the development of malignant neoplasms. Drugs in this group include:

Nimustine;
Cisplatin;
Chlorambucil;
Busulfan.

It is important to remember that all drugs have contraindications and can lead to adverse reactions. Therefore, they can be used only as prescribed by the attending physician, without exceeding the dosage.

In some cases, particularly large lesions are removed through surgery.

Besides pharmaceuticals Physiotherapeutic procedures are used to treat pigmentary urticaria. Most patients are indicated for PUVA therapy sessions.

The method consists of external application or orally psoralens: medications plant origin and subsequent irradiation with ultraviolet rays. In this case, individual places or the entire body are exposed, depending on the location of the rash.

As a result of complex procedures, the process of proliferation of mastocytes is blocked, rashes are stopped or completely disappeared, and redness and peeling of the skin are eliminated.

Signs of improvement are observed after 5-6 procedures, and the period of remission can last from 6 months to several years.

PUVA therapy is contraindicated in the treatment of infants, individual intolerance to UV radiation, or suspected malignant disease.

Forecast

With the dermatological form of mastocytosis in young children, doctors give a favorable prognosis. Most often, by the age of 13-16, all symptoms of the disease disappear without special therapy.

In the systemic form, the most dangerous is mast cell leukemia, which is malignant process, having 95% death. Other types of systemic mastocytosis also have an unfavorable prognosis because they lead to dysfunction of internal organs

Prevention

Since mastocytosis is a poorly understood disease, the mechanism for its prevention is not well developed. Therefore, the main preventive measure is to prevent the proliferation of mast cells and their release of histamine and other mediators. To do this, you must follow these recommendations:

observe special diet, excluding allergenic products;
avoid hypothermia;
not be there long time under the scorching rays of the sun;
do not take medications without a doctor’s prescription;
stop smoking, drinking alcohol and other bad habits;
in any situation, maintain calm and peace of mind;
visit more often fresh air, do physical exercise.

In addition, it is important to take care of your health: undergo regular medical examinations, treat even the most minor diseases, for any unpleasant sensations or symptoms, consult a doctor.

Urticaria pigmentosa is one of the most common forms of a rare disease called mastocytosis. In many cases, children are susceptible to this disease, but adult women and men can also get it.

Mastocytosis is a disease in which mast cells multiply and accumulate in living tissues. Mast cells are a fairly significant part of the human immune system, so many experts are confident that urticaria pigmentosa appears when the body’s immunity is impaired.

Children most often suffer from urticaria pigmentosa; spots appear on their body, red or pink, and later they change into subcutaneous blisters, inside of which there is water. After they heal, traces of a brownish tint remain on the body. Sometimes they disappear, and sometimes they remain and may even increase in size.

In adults, urticaria pigmentosa begins to occur in the form of spots that have a clear border and are absolutely smooth; nodular formations no larger than 5 mm may occur, and there may be rashes in the form of blisters. These inflammations can be seasonal or appear from any damage to the skin, sometimes they are with a person throughout his life, then disappearing, then appearing again.

The symptoms that characterize this disease directly depend on where exactly, in which layers of the epidermis, substances released from mast cells have accumulated.

Doctors are used to dividing the skin reactions that occur with urticaria pigmentosa into five types:

Maculopapular. Many small nodules or dark spots form on the skin. Localization is clearly limited. If you apply mechanical force to them, small bubbles may appear.
Nodal. With this type, many convex seals or nodules appear on a person’s skin. The color of the formations that appear is usually red, pink or yellow. Sometimes the nodules, due to their close proximity to each other, merge into plaques.
Solitary. A knot no larger than 50 mm in size is formed; when you touch it, you might think that it is rubber. These nodes are called mastocytomas. Their localization is usually on the arms or neck, on the torso. Usually only one nodule appears, but it is possible to increase their number to four. They have the ability to disappear on their own.
Telangiectatic. This type usually affects women in adulthood. They have red-brown spots on their body, mainly on their chest.
Erythrodermic. Yellowish-brown areas appear on the human skin, quite dense, they have uneven edges, but the border is clearly visible. With this form of the disease, a person suffers from severe itching, which, as a result of scratching the affected areas of the body, leads to small ulcers and cracks.

At little impact Blisters may appear and the itching may become worse. There is a risk of developing a bullous form, when there are a lot of bubbles. Transformation into systemic form occurs much less frequently, but then the entire skin will be affected, will peel and be very difficult to treat.

In addition to the signs that appear with urticaria pigmentosa on the skin, the following symptoms may appear:

the human body temperature rises;
blood pressure decreases for unknown reasons;
strong itchy skin and redness of the skin;
attacks of tachycardia.

These symptoms may indicate that the disease may have damaged a person's internal organs. And in this case, it already becomes quite life-threatening and requires good and professional treatment.

Forms of mastocytosis

There are several forms of mastocytosis:

systemic. It usually appears in adults, and affects the tissues of the internal organs of a person, but the lesions skin may or may not be;
cutaneous This form includes both infantile and adult mastocytosis. With this form, in infancy and adolescence, only the skin is affected; the disease does not spread to the tissues of internal organs. Cases of transition to the adult form are very rare;
malignant. It transforms mast cells into malignant form. There may not be inflammation on the skin; it only affects the internal organs. Difficult to treat and can lead to death.
adult or teenage form. It manifests itself as symptoms on the skin, but also affects the internal organs of a person, the heart, kidneys, and stomach. Although such lesions rarely progress and become systemic.

Treatment of urticaria pigmentosa

For this disease, treatment must be prescribed by a doctor; it is individual and depends on many factors:

symptoms;
age of the sick person;
causes of the disease.

When does mastocytosis affect children, but severe symptoms is not on the skin, then no therapy is prescribed, since everything goes away on its own adolescence. When cases are more complex, drug treatment is prescribed.

Antihistamines, such as:

Cetirizine;
Suprastin;
Tavegil;
Diazolin et al.

Anti-bradykinin drugs such as:

Anginin;
Prodectin, etc.

Antiserotonin drugs such as:

Ketotifen;
Bicarfen;
Periactin et al.

Corticosteroid drugs, drugs based on cromoglyceric acid, such as:

Intal;
Nalkrom;
KromHexal et al.

Drugs from the group of cytostatics:

Prospidin et al.

PUVA therapy may also be prescribed. This method involves exposing the skin to photoactive substances, medicinal purposes and ultraviolet rays. If the symptoms on the skin are very pronounced, they can be removed through surgery.

The treatment prescribed by the doctor will always depend on the form of the disease. And in each case, the approach will be different. Therefore, at the first sign of this disease you need to see a doctor urgently. He will hold necessary tests and research, and, having determined the form of mastocytosis, will be able to make an accurate diagnosis and prescribe appropriate treatment.

You should always follow the treatment regimen prescribed by the doctor; you should not prescribe or use drugs on your own that the specialist did not mention. If there is a definite need to find out about the drug that you want to use, then it is better to first ask your doctor about it, in case it is not suitable for you at all. And you will not only spend extra money, but you can also worsen your situation.

Traditional methods of treatment

Treatment of urticaria pigmentosa, in addition to medication and physiotherapy, also includes adherence to a special diet. With such a diet, all salty, fatty, smoked and spicy foods are prohibited.

It is advisable to steam foods or eat them boiled. It is imperative to exclude soda and drinks containing alcohol from drinks. Fans of spicy seasonings and sauces will also have to give them up.

You can also resort to old traditional methods of treatment, but you must first consult with your doctor. For example:

Nettle infusion. Add a spoonful of nettle leaves to a glass of boiling water and let it brew for no more than thirty minutes. And then drink 4 or 5 times a day, half a glass of warm infusion.
Celery juice. This method is not suitable for everyone, since celery has a rather specific taste, but if you don’t mind trying it, you should drink the juice three times a day, half a glass before meals.
Beetroot infusion. Fresh beets, peel, cut into small pieces and add three liters of water. Keep this in a dark place for no more than six days. Next you need to drink one tbsp. l. three times a day. You can also apply compresses to the affected areas of the body.
Potato compress. This kind home treatment It occurs quite often and helps with many skin inflammations, soothes it, and relieves itching. You need to take fresh potatoes, grate them and, wrap them in gauze, apply the resulting compress to the inflamed and affected areas of the skin for no more than forty minutes.
Baths from herbal infusions. You can also take medicated baths to soothe your skin. To do this, you need to mix celandine, chamomile, valerian, sage and string in equal parts, pour everything with plain water and boil for about 10 minutes. Next, it is infused for about half an hour, and then the resulting infusion must be added to a bath filled with warm water. It is not recommended to take such a bath for more than 10 minutes.

Disease prevention

People who have been diagnosed with any form of mastocytosis should follow preventive measures, such as:

Increase your immunity by taking vitamins or undergoing special therapy;
Follow the diet prescribed by your doctor;
Eliminate or replace certain medications that must be notified to your physician.
Be sure to treat inflamed areas of skin with caution, do not injure them or apply mechanical stress.

Sometimes, in adults, urticaria pigmentosa sometimes disappears and then appears again; they need to very carefully monitor the symptoms of the disease, and at the first signs of its relapse, go to the doctor.

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. The following forms of mastocytosis are distinguished:

The American classification provides for the division of urticaria pigmentosa into the following forms:

isolated non-aggressive:
A. asymptomatic
b. cutaneous
V. systemic with visceral manifestations
mastocytosis with hematological disorders:
A. myeloproliferative
b. myelodysplastic
aggressive
mast cell leukemia

Clinical symptoms of urticaria pigmentosa are characterized by multiple spots and papules of irregular round shape from yellowish-brown to reddish-brown. This is due to the presence of melanin in the epidermis above the clusters of mast cells. Mechanical irritation of such areas leads to the appearance of redness and a blister on the skin (Darier-Unna phenomenon).

The disease usually appears during early childhood and often disappears spontaneously after a few years during puberty. The course of the disease is usually benign. But approximately 30% of patients periodically experience hyperemia, itching, and urticarial elements; less than 15% experience vomiting, deep fainting, and shock. 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.

The diagnosis is confirmed by a morphological examination of a skin biopsy, which reveals an increase in the number of mast cells by 10 times compared to the norm. Used for treatment large doses intala orally. Antihistamines are ineffective.

We observed a patient with a combination of urticaria pigmentosa and anaphylactic shock on novocaine. Here is our observation. Patient Z., 68 years old, 02/12/1999 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 treated for anaphylactic shock following the administration of novocaine. From January 18, 1999 to 02/08/1999 the patient was treated in the proctology department of the 2nd city hospital, where she underwent a polypectomy under local anesthesia lidocaine. Before discharge, novocaine was injected into the area anus. After 15 minutes, the patient felt weak and lost consciousness. Assistance was provided in the intensive care unit of the 3rd city hospital.

History of the disease. The patient's pigmented rash had been observed since 1945, when before deportation to Germany, Z.'s skin was treated with some kind of ointment. Then a rash developed, followed by pigmentation. She did not consult a doctor about the rash and did not receive treatment.

Anamnesis of life. From past diseases notes appendectomy in 1984. Blood pressure periodically increases for about 10 years. Takes clonidine. He does not notice any pain in the heart. There are two children (physiological birth).

Allergy history. In 1984, after an appendectomy in the hospital, while taking some medicine in a tablet (sulfonamide?), I developed severe weakness. There were no reactions to food products. Among the relatives of patients allergic diseases No.

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

Visible mucous membranes are pink in color without pathological elements. The lymph nodes, accessible palpations are not enlarged. The isthmus is palpated thyroid gland. The mammary glands are soft and painless. In the lungs, breathing is vesicular. Heart sounds are muffled, rhythmic, heart rate - 60 per minute. The abdomen is soft and painless. The liver and spleen are not enlarged. Stool and urine output are normal.

General blood test (02/15/99). red blood cells -4.0 x1012 / l; hemoglobin - 125 g/l; leukocytes - 7.2x109 /l; n - 1%, s - 60%, e - 9%, l - 29%, m - 1%; ESR - 3 mm/h.

Biochemical blood test (02/12/99). Urea - 8.7 mmol/l; ALT - 0.14 µmol/h H l; AST - 0.23 µmol/h Ch l; K - 4.08 mmol/l; Na - 141.2 mmol/l; CL - 102 mmol/l; total protein- 85 g/l.

General urine test (02/11/99). 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 - crystals uric acid. No worm eggs were found in the feces (02/17/99).

ECG (01/18/99). 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 (02/16/99). VOD - 0.9; VOS - 0.7. Fundus - discs are pale pink, clear boundaries; retinal vessels of normal caliber. Examination by a neurologist (02/18/99). No neurological pathology was detected. Conduct morphological study A skin biopsy was impossible because the patient had an anaphylactic reaction to novocaine.

Considering the long history of increased 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 about the paroxysmal release of histamine by mast cells and a reflex effect on the activation of the adrenal medulla with the release catecholamines. Noteworthy is the combination of urticaria pigmentosa and anaphylactic shock, which can lead to a condition that is difficult to treat.

Urticaria pigmentosa is called mastocytosis. The disease occurs in children younger age. It also occurs among the adult population. The disease is associated with impaired pigmentation of the skin.

Urticaria pigmentosa is associated with a disorder 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 in close relatives.

According to the second theory, mastocytosis occurs when the immune system fails. Patients experiencing inflammatory processes are more likely to suffer from urticaria pigmentosa. Mastocytosis also occurs without an obvious cause.

Urticaria occurs when excess mast cells accumulate in tissues. Their destruction leads to the release of histamine, heparin and other substances. They increase vascular permeability. Tissue swelling occurs. The capillaries expand. A change in the skin occurs. Arterial pressure goes down. Body temperature rises, tachycardia develops. Symptoms are accompanied by severe itching.

A number of factors influence the increase in histamine:

Urticaria pigmentosa, or mastocytosis, gives the inflamed areas 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, bones and internal organs are affected.

Under mechanical influence pigment area sticks out 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. The burning sensation causes the blisters to scratch and become damaged. Inflammations change shape, increase or disappear over time.

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

Symptoms persist for 2-3 days. After this, the signs of the disease disappear. Systemic mastocytosis is more serious. Patients experience:

allergic reaction;
breathing problems;
decrease in pressure;
bleeding, cramps;
pain syndrome in bones and muscles.

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

Experts cannot yet pinpoint the exact cause of urticaria pigmentosa. Some argue that it is a matter of heredity; someone - that in decreased immunity.

How does mastocytosis occur in children?

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

The formations are localized on the patient’s body, legs and arms. Rarely, rashes appear on the face. Nodules and brown plaques form on the affected areas.

The disease is not accompanied by any malaise in the child. Fever, increased temperature, and 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 based on 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 a comprehensive blood test. It is carried out to exclude the systemic form of urticaria. Treatment is symptomatic.

Forms and types of disease

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

Cutaneous mastocytosis in infancy. The rash appears in infancy. Recurrence is possible during puberty. The disease does not develop. There is no system component.
Urticaria with systemic changes. Changes occur in internal organs. The disease does not develop. IN childhood pathology regresses. In elderly patients, the disease becomes systemic.
Systemic mastocytosis. Leads to damage to internal organs. Develops against the background of external signs of the disease.
Malignant mastocytosis. It is called mast cell leukemia. Determined by the presence of malignant mast cells in organs, tissues and bone skeleton. External signs there are no manifestations. Possible death.

Mastocytosis is also classified according to the type of skin manifestation.

Nodal type. It occurs with the formation of nodes and tubercles, with a smooth or bumpy surface. The color of the formations is yellow, red, pink. Different in tone from healthy skin. The number of nodes varies with the degree of disease. The rashes unite into single areas.
Maculopapular type. It is characterized by the appearance of dark-colored nodules with smooth contours. Mechanical impact leads to the appearance of bubbles. Externally similar to urticaria.
Infant type. It is called solitary mastocytosis. Associated with the appearance of nodes up to 50 mm in size. The skin on the node is dense. Location of nodes: neck, body, arms. In infants, there is predominantly one mastocytoma. Rarely up to 4 pieces. Formations are characterized sudden appearance and disappearance.
Erythrodermic type. 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 scratching, the inflamed areas are injured.
Telangiectatic type. Belongs to the category of persistent urticaria pigmentosa. Rarely occurs in patients. It appears in adulthood in women. Characterized by spots of red and brown color. The nodes are localized on the patient’s chest, legs and arms.

Features of diagnosis and treatment

Mastocytosis has many causes and forms of manifestation. Only qualified specialist can identify the causes and prescribe the right treatment.

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

In severe situations, symptomatic therapy is prescribed:

taking antihistamines (Diazolin, Tavegil, Zodak, Zirtec, Suprastin);
use of antiserotonin drugs (Ketotifen, Bicarfen);
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 medications at the same time. Medicines promote the penetration of radiation deep into the epidermis. Surgery used when conservative treatment is ineffective.

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

Infantile mastocytosis resolves to puberty. In adulthood, cutaneous mastocytosis progresses. The disease affects internal organs and leads to death.

Mastocytosis: what is it? Urticaria pigmentosa (English: urticaria pigmentosa, ICD-10 code Q82.2 Mastocytosis, L50.8 Another 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 large number of mast cells(another name is mastocytes) in the skin. Their peculiar the job is to inflame foci of inflammation They release a chemical called histamine in response to germs and other viruses.

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

This is a 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. In this condition, 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 spots, which look like freckles and/or bumps (papules) that appear on the skin. IN in rare cases 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 surrounding healthy tissue.

This reaction is known as Darier's sign(used for diagnostics). These areas, most commonly referred to as blisters or welts, tend to be very itchy and can change in size and shape over the course of several hours.

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

Other symptoms may include:

Hyperemia (redness of the skin).
Change in color of spots to dark brown or black.
Severe diarrhea.
Tachycardia (rapid heartbeat).
Nausea or vomiting.
Fainting or fainting.
Dizziness and headache

Reference! Rarely, against the background of urticaria pigmentosa, adult patients may develop telangiectasia, which is an expansion of the superficial vessels of the skin.

In most cases symptoms urticaria pigmentosa lasts 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 occurs 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 can occur in other forms of mastocytosis such as aggressive systemic mastocytosis.

Causes

Exact reasons occurrence of urticaria pigmentosa not yet studied. In some cases it occurs without visible reasons, sometimes a specialist assumes that it was inherited as an autosomal dominant genetic trait. However, not all children with the defective gene will have symptoms of the disease.

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

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

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

Factors activating increase mast cell concentrations:

Physical stimuli (heat, friction).
Excessive physical activity.
Bacterial toxins (as well as poisons).
Poisoning of the body.
Eye drops containing dextran.
Alcohol.
Some foods: lobster, crayfish, fish, cheese, lobster, very hot drinks, spicy foods.
Abuse of painkillers.
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 at 15-30% in adults with urticaria pigmentosa.

Urticaria pigmentosa: photo

Systemic mastocytosis: photo

Diagnostics

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

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

The following may also be prescribed tests How:

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

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

Most often, skin lesions result from 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;
actinic keratosis (seborrheic).

Seeing a doctor if you suspect urticaria pigmentosa inevitably.

Ambulance may be needed if:

The patient has difficulty breathing and wheezing is heard.
Swelling of the throat or tongue has appeared.
The patient notes a constant feeling of weakness, there are symptoms such as heat or chills.
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

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

Immediately stop taking medications such as:

aspirin (not dangerous in small doses);
codeine.

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

Medication

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

Mastocytosis in adults, treatment with drugs:

H1-antihistamines, used to relieve symptoms such as itching and flushing, as well as H2-antihistamines– to treat 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 of the release of histamines; in this case, the patient must inject himself injection solution adrenaline (epinephrine).
This course of action is necessary to avoid circulatory collapse and shock.
Mast cell stabilizers, such as sodium cromoglycate, promote degranulation of mast cells after exposure to specific antigens. These agents are good against diarrhea, abdominal pain, headaches and bone pain.
The course of treatment with such drugs is usually about a week.
Low dose aspirin may also help, although in some cases it may worsen. Treatment with low doses of aspirin is indicated patients with acute vascular insufficiency.
Photochemotherapy. If urticaria pigmentosa appears, treatment is used with long-wave UVA radiation (340-400 nm). In irradiated skin, a decrease in the concentration of mast cells is noted. Course of treatment: 2-3 procedures every week for several months.
Photochemotherapy reduces itching and improves appearance skin.
Steroid drugs and creams are used to treat systemic mastocytosis.
Recently, therapy with interferon and imatinib has been used to treat severe forms, as well as for the treatment of systemic mastocytosis ( anti-leukemia drug).

Examples medicines are presented below.

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

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

Ketotifen's analogs: AllergoKomod, Dipolkrom, Kromohexal.

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 daily for 2 to 3 weeks) may also reduce symptoms.

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

Often performed for cosmetic reasons removing dark age spots . This is a very expensive but painless procedure. The price usually depends on the diameter of the spot; removing a 1 cm spot costs about 2000 rubles.

Folk remedies

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

Diet

For urticaria pigmentosa you should adhere to the following rules:

Eliminate alcohol completely.
Eliminate spicy and fried foods from your diet.
Add organic oils to your diet - coconut or olive.
Drink plenty of water, at least 1.5 liters per day.
Reduce your intake of saturated fat (dairy) and increase your intake of omega-3s (nuts, avocados).
Eat enough fiber (whole grain bread).
Reduce your sugar and salt intake.
Eat fresh fruits and vegetables.
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 serious illness, which may affect various organs, in any case, independent treatment of the disease is not recommended.

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