Pemphigus in adults. True pemphigus

Pemphigus vulgaris is considered one of the diseases that has an autoimmune mechanism of development. It is characterized by the appearance of bubbles on the mucous membrane and dermis. If these are opened, pink wounds will appear in their place.

So, let's learn more about the symptoms and causes of pemphigus vulgaris (ordinary) disease, its treatment in adults, children and newborns.

Features of the disease

Pemphigus vulgaris is known in the medical community as the most popular form of pemphigus. More often, the disease occurs in older patients whose age ranges from 30 to 60 years.

The first manifestations are noted on the mucous membrane lining the oral cavity. After some time, the disease spreads to the dermis. If bubbles appear on the genitals of women or in the mouth, they quickly burst.

We will discuss below the reasons why pemphigus vulgaris may appear in the medical history.

Pemphigus vulgaris (photo)

Causes

Typically, this disease occurs when the immune system is not functioning properly. In this case, the body produces antibodies (IgG) to its own cells, which are localized in the spinous layer of the epidermis.

Desmosomes, which bind epidermal cells, are destroyed due to the influence of autoantibodies. When connections between cells are lost, spaces filled with intercellular fluid appear. This is how acantholytic blisters are formed.

The video below will tell you about the signs of pemphigus:

Symptoms

Often, the development of pemphigus begins with the mucous membranes (mouth, pharynx). It is very difficult to detect them in a timely manner, because these bubbles burst very quickly. After their accidental opening, only those that remain are painful and have a characteristic bright red color. If treatment is not started, the bubbles grow and merge. At this stage of the disease, the following symptoms are observed:

foul odor from the mouth;
decreased appetite due to pain;
erosions on the mucous membrane oral cavity.

Bubbles will begin to appear on the epidermis several months after their formation on the oral mucosa. Very rarely it can be observed around the bladder. It is like a thin rim. Rashes with this pathology are focal in nature. The rash usually appears in the following areas:

inguinal folds;
back;
axillary areas;
breast.

The opening of the bubbles occurs several days after their occurrence. The resulting erosions are distinguished by their bright pink color, large size, and tendency to merge. The patient begins to worry about the following signs:

the appearance of purulent discharge on erosions;
pain;
cloudiness of the liquid accumulated inside the bubbles;
(it can develop after infection).

Diagnostics

Mechanical symptoms indicating acantholysis are considered particularly significant. Specialists can perform the following procedures:

Detection of Nikolsky's symptom. This symptom consists of peeling of the epidermis after slight rubbing of the healthy dermis.
Detection of marginal Nikolsky symptom. To do this, you need to pull a piece of skin from the burst bubble. The symptom will be positive if the epidermis peels off at a significant distance from the erosion.
Detection of Asbo-Hansen's sign. To do this, you need to press your finger on the bubble. The answer will be positive when the epidermis peels off in the area of the periphery of the bladder and its area increases.

To confirm the suspected diagnosis, you can carry out cytological examination(Tzanck method). Thanks to microscopy, a smear taken from the bottom of the wound can be detected. These cells are present in the stratum spinosum of the epidermis. They take material from a fresh bubble.

They can also conduct immunological studies (direct/indirect RIF). They are necessary to confirm/refute the autoimmune nature of the disease.

Treatment

The only one effective method Treatment for this disease involves the use of medications. As an auxiliary method, you can use a therapeutic one.

Therapeutic

Along with the use of medications, extracorporeal hemocorrection is prescribed. To purify blood, the following are most often used:

plasmaphoresis;
hemosorption.

Medication

Drug therapy involves the use of the following groups of drugs:

corticosteroids ("", "Triamcinolone", "");
cytostatics (“ ”, “ ” “Azathioprine”).

Antibiotics are also needed in case of infection. In order to prevent complications that may arise as a result of corticosteroid therapy, it is necessary to take medications that have a protective function on the walls of the stomach (“Bismuth Nitrate”).

You should not treat pemphigus with folk remedies without your doctor’s permission!

In this video, Elena Malysheva will talk about the treatment of pemphigus:

Prevention of pemphigus vulgaris

After eliminating the signs of the disease, you should think about a number of preventive measures which are necessary to prevent relapses. They are:

monitoring the condition of the dermis;
taking vitamins, calcium, potassium;
monitoring the manifestation adverse reactions after taking medications;
control (regular) sugar levels in urine and blood;
control over prothrombin.

Pemphigus vulgaris in the oral cavity

Complications

Because of large quantity side effects glucocorticoids can cause serious complications. Long-term use of these drugs may cause:

atrophy of the adrenal glands, cessation of the body’s production of its glucocorticoids;
failures in carbohydrate metabolism, in addition to this, the occurrence of steroid diabetes;
changes in mental state (emergence of euphoria, manic-depressive);
exacerbation (peptic);
thrombophlebitis;
disruptions in the menstrual cycle;
disruptions in protein metabolism;
slowing down recovery processes;
violation of fat metabolism;
spontaneous fractures (they occur due to bone decalcification);
decreased immunity.

Forecast

If treatment is started promptly (using corticosteroids), death can be avoided. The use of drugs in this group can provoke the development dangerous complications in area internal organs, systems.

The patient will have to take corticosteroids throughout his life, but in small dosages. Long-term use of such medications can also cause death.

After 35 years, some men and women may develop a disease called pemphigus. The disease affects the skin or mucous membranes of a person. Although most often it is not possible to notice signs of inflammation, the skin becomes covered with large blisters - bullae. Without timely treatment they merge into larger formations, capturing more and more space.

Unfortunately, the exact causes of the disease have not yet been established. But it is known that pemphigus is a disease with many faces. It is believed that it may be associated with a viral nature or disturbances in water-salt metabolism, or improper protein metabolism in the body. Some experts believe that this is due to a defect in the cell membranes that maintain contact between cells. Probably, it is the causes of the occurrence that determine the course of the symptoms, which may vary. They depend on the form of the disease, its “face”.

This is how it affects healthy-looking skin with blisters. Transparent formations may be filled with yellowish or bloody fluid. When they burst, scabs or ulcers form. Even after complete healing on the patient’s body for a long time brown spots remain. The disease is accompanied by severe burning and itching.

Pemphigus vegetans occurs in a more severe form. During this disease, large blisters may appear in the mouth, around the navel, in the armpits, on the genitals, in anus. The blisters that appear are very painful, they interfere with the normal functioning of the body, making it difficult to swallow, stool, and urinate. After the blisters are opened, a lot of pus is released from them, and extensive, long-healing erosions remain on the body.

It is localized on the face, resembling a butterfly in its shape. Bubbles with this type of disease do not appear immediately. A rash that appears on the face can move to the shoulder blades, stomach, and other parts of the body over the course of several months, causing severe itching, peeling, and swelling. Bubbles may appear after a long time, and then the form of the disease becomes outwardly indistinguishable from pemphigus vulgaris.

The leaf-shaped form of the disease, in which the blisters are sluggish and quickly open, can lead to an increase in temperature. The peculiarity of this form is that skin ulceration affects very large areas of the body.

In children, the most common form of the disease is viral pemphigus, which they become infected with at any time of the year. The causative agent is an enterovirus, which spreads very quickly through coughing or sneezing or contact with contaminated stool. Although the disease is considered harmless, it can cause swelling in the mouth and limbs. Usually the symptoms disappear after a week, but to ensure that the consequences do not affect the overall illness, it is recommended to show a doctor.

How is pemphigus treated? The disease requires an integrated approach. Typically, dermatologists prescribe a combination treatment with hormonal drugs (most often prednisol) and external ointments, for example, betamethasone. In addition, it is recommended to take cytostatic agents. The disease usually responds well to this treatment. However, in most difficult cases Plasmapheresis or blood purifying procedures may be required. Dermatologists recommend that patients follow a diet that excludes any food that has irritating effects.

How dangerous is pemphigus? A disease that is diagnosed very late may progress to a protracted stage, in which it will affect most of patient's body. Sometimes deaths occur.

A chronic disease of an autoimmune nature, which manifests itself through the formation of blisters on the skin and mucous membranes, is called pemphigus. This pathology has several stages of progression.

The child's body is fragile and susceptible to many diseases. A disease in which not water but purulent blisters form on the child’s body is called streptoderma. You can read more about this disease in the article on the topic of streptoderma in children, photo.

Symptomatic manifestations of the disease:

blisters in the mucous membranes of the eyes, mouth or genitals;
the appearance of an unpleasant odor in areas of affected skin;
the formation of colorless bubbles inside;
after the vesicles rupture, ulcers appear.

Most often, signs of the disease are localized on the mucous membranes in the area:

groin areas;
nasal cavity;

Bullae are lesions or certain sacs under the skin, limited by the epidermis and filled with erosive fluid. They are similar to others skin rashes- vesicles and blisters.

The main difference is only in the size of the bullae. By the way, they reach at least 1 cm in diameter.

The size of the bubble is even larger in case of frostbite or burn.

The bulla itself consists of several specific layers:

Upper leather. The most thin part, very often serous fluid is visible through the septum. The layer is sometimes called the "tire".
Cavity with liquid.
The deep layer of skin that forms the “bottom” of the bladder.

Mechanism of bubble formation

Human skin can be figuratively described as a water-spring “mattress” covered with a kind of “wall”. The “mattress” does not participate in the formation of bubbles - only the top layer, the epidermis, suffers.

On top of the “wall” there are layers of cells that are no longer quite similar to cells, reminiscent of applied cream. These are scales, corneocytes, necessary for protection from mechanical, chemical and physical damage.

Causes of pemphigus

Reproduction of microorganisms under the dermis

The most likely cause of pemphigus is a disorder of autoimmune processes, as a result of which the body's cells become antibodies to the immune system. Violation of the antigenic structure of epidermal cells occurs under the influence external factors, in particular exposure to retroviruses and aggressive conditions environment.

The damaging effect on epidermal cells and the production of specific antigens leads to disruption of communication between cells, resulting in the formation of blisters. Risk factors for pemphigus have not been established, but in individuals with a hereditary predisposition, the incidence rate is higher.

Possible root causes of pemphigus formation are disturbances in the functioning of the immune system. child's body. As a result, the immune system reacts to its own cellular structures.

But damage to the integrity of the skin occurs under the influence of retroviruses or aggressive conditions external environment. Bubbles are formed due to disturbances in metabolic processes between cells.

The main factors provoking the disease are:

diseases of the nervous system;
violation metabolic processes body;
diseases of endocrine organs;
changes in the structure of enzymes;
exposure to harmful factors.

This lesion of the dermis is characterized by an autoimmune mechanism of development, in which the appearance of autoantibodies to skin cells is observed. The disease is very dangerous (possible death) due to cachexia and secondary infection.

Until now, scientists have not established the reasons that provoke changes in the immune system. There are versions about the influence of exogenous factors on the body (if the patient has genetic predisposition).

Types and stages of pemphigus

Bullous dermatitis can be divided into several categories:

We will talk about the diagnosis and treatment of bullae and water blisters on the body in adults and children below.

Pemphigus is a fairly common disease, since one of the varieties of its forms is viral. A sick person can easily infect a healthy person who has a weak immune system during this period. The incubation period is only 3 to 6 days. Both men and women are equally likely to get sick. Depending on the stage of development of the disease, there are 4 main stages of pemphigus:

initial stage - characterized by multiple rashes in the form of blisters with clear liquid, on no more than two parts of the body;
stage of active spread of the disease (generalization) - the general condition worsens, signs of dehydration are recorded, rashes appear on three or more anatomical areas of the body;
temporary weakening or disappearance of the main symptoms, in particular, after a course of glucocorticosteroids, which have an immunosuppressive effect;
repeated exacerbation of pemphigus - observed in the chronic, most common form.

Pemphigus as a dermatological disease has not been fully studied to this day. Doctors and scientists cannot determine the main reasons for its origin, but they have already been able to accurately identify two main varieties: acantholytic or true pemphigus and non-acantholytic or benign pemphigus.

Each of them is divided into several subspecies. Thus, the acantholytic form is divided into 4 key types:.

Vulgar is the most common. Blisters, as the main symptom of the disease, are localized on the back and chest, as well as on the oral mucosa. In this case, the initially formed single foci gradually spread throughout the cavity and can merge with each other. After opening the bubble, a bright red erosion forms. Severe pain makes it difficult to eat.

Symptoms of the disease

There are several main types of pemphigus, and each of them has its own symptoms.

Pemphigus vulgaris

Photo: pemphigus vulgaris on the forearm

This form of pemphigus is characterized by the presence of blisters throughout the body. Their shell is thin, sinks in the center, and the purulent contents are cloudy. Bubbles appear first in the oral cavity and cause an unnecessary visit to the dentist.

Pemphigus in adults is a chronic disease with an undulating course, that is, it is characterized by alternating periods of extinction of clinical manifestations and exacerbations of the disease. A characteristic sign of the disease is the appearance of blisters (bulls).

Bubbles can be localized on the mucous membranes of the mouth, upper respiratory tract, external genitalia, and skin. There are several forms of pemphigus:

Ordinary (vulgar);
Vegetative;
Leaf-shaped;
Erythematous (seborrheic);
Brazilian.

Pemphigus vulgaris

This is the most common form of pemphigus in adults. It usually begins unnoticed, for no apparent reason.

The disease manifests itself with the appearance of blisters on the mucous membrane of the mouth, red border of the lips, nose, and nasopharynx. The patient experiences pain when swallowing food and saliva, and when talking.

In addition, increased salivation is noted and, characteristically, bad smell from mouth. Patients often turn to a dentist or otolaryngologist with such symptoms and are unsuccessfully treated for stomatitis, rhinitis or laryngitis.

Initially, areas of skin with specific redness appear on the patient’s body, which are covered with blisters (flatten, flabby). Sometimes the disease can develop like pemphigus vulgaris, Dühring's dermatitis, and other types of dermatitis. In some cases, the bubbles are very faint.

Whether a person has become infected or not after contact with a sick person will not be visible immediately, but after 3-10 days. incubation period. Next, children develop general signs, indicating that the child is sick:

weakness;
fast fatiguability;
drowsiness;
loss of appetite;
may be: runny nose, sore throat, headache, cough, sometimes loose stools.

Pemphigus is divided into several types: viral, common, vegetative, foliaceous and seborrheic.

Viral pemphigus is a common, harmless skin disease caused by an enterovirus. Most often, such pemphigus is observed in children in autumn or spring and goes away after a week.

Infection occurs, for example, during sneezing, and symptoms appear after a few days. Manifestations of the disease can be seen in the mouth (making it difficult to eat) and on the extremities.

Thin-shelled blisters appear that may rupture. The child feels weak and has a fever, and may have a sore throat.

No special treatment is required, except for treating wounds with disinfectants and excluding spicy and spicy food to avoid irritation of the mucous membrane.

Pemphigus vulgaris begins acutely and, as a rule, begins with damage to the oral cavity. This symptom is the only manifestation of the disease for a long time.

The patient observes the appearance of single bubbles or a small number of them in the tongue area. Due to mechanical damage, the shell of the bubbles is gradually damaged and opened, forming bright red erosions.

They are so painful that a person cannot chew and swallow food. Later, deep cracks appear in the corners of the mouth, which further complicate the course of the disease.

After 3-5 months, blisters appear on other parts of the body. They can be of various sizes, with serous or cloudy contents.

The rash covers increasingly large areas of the skin, forming large lesions. Opened blisters leave painful erosions, and later secondary pigmented spots.

Scars form rarely and only against the background of associated infection or damage to the basement membrane.

Often, the development of pemphigus begins with the mucous membranes (mouth, pharynx). It is very difficult to detect them in a timely manner, because these bubbles burst very quickly. After their accidental opening, only erosions remain, which hurt and have a characteristic bright red color. If treatment is not started, the bubbles grow and merge. At this stage of the disease, the following symptoms are observed:

foul odor from the mouth;
decreased appetite due to pain;
erosions on the oral mucosa.

Bubbles will begin to appear on the epidermis several months after their formation on the oral mucosa. Very rarely, redness of the dermis around the bladder may be observed. It is like a thin rim. Rashes with this pathology are focal in nature. The rash usually appears in the following areas:

inguinal folds;
back;
axillary areas;
breast.

Traditional treatment

Bubbles that form in the active phase of the disease are located inside the epidermis. At the same time, the skin around remains unchanged.

They have a very soft and thin shell through which a transparent liquid can be seen. If it turns whitish, it means there is a bacterial infection.

After a few days, erosion forms in the focal lesions, and the bubble opens. It has been repeatedly noted that the patient emits a specific smell, similar to rotten apples.

To confirm the diagnosis, you can conduct a kind of experiment: if you pull the membrane of the bladder or move two areas of skin near it, a detachment of the epidermis by 1 - 2 millimeters will become noticeable.

Large bubbles may take on a pear shape due to the gravity of the contents. Soreness of pemphigus foci is not always noted, as is itching.

However, the resulting erosions always cause a lot of discomfort. Because of inflammatory process the ulcers are bordered by a red rim, then covered with a crust.

It disappears on its own after a few days.

The symptoms described above appear approximately 3-4 days after the end of the incubation period. The duration of which is from 3 to 6 days. The first signs indicating the initial stage of the disease are:

general deterioration of condition, weakness;
increased body temperature;
deterioration of mood and appetite;
rarely - cough, runny nose, migraine.

When rashes occur in the oral cavity, pain syndrome inevitable. The pain intensifies when the blisters come into contact with cold, hot, sour and spicy foods.

The condition becomes especially severe after opening of the formations. Often, a rash in the mouth causes nausea and vomiting.

In most cases, the cervical and submandibular lymph nodes are enlarged.

If the pemphigus rash is localized on the extremities: fingers, hands, feet, a few days after the onset of the disease, the nails are most likely to begin to crumble and peel off.

Interestingly, this symptom does not cause painful sensations. After 2-3 weeks, a new nail plate grows, so there are no external traces of the transferred pemphigus.

The main symptom of this disease is blisters that appear throughout the body on healthy areas of the skin and mucous membranes. Their size rarely exceeds three centimeters in diameter.

At the initial stage, pemphigus disease manifests itself through white or transparent rashes, which become cloudy and filled with blood over time. In some cases, the contents of the blisters spill out, but most often they dry out, forming a crust of the contents.

Diagnostics

A dermatologist diagnoses and treats skin pathologies. On external examination, pemphigus is early stages It is difficult to suspect, so the patient undergoes biochemical tests:

Blood analysis
The platelet count determines the general state of health and immunity.
Analysis of urine
An increased content of leukocytes and protein breakdown products indicates the presence of an extensive inflammatory process.

Laboratory technicians culture a urine sample into a culture medium. The pathogen begins to actively reproduce with the formation of colonies. Pemphigus enterovirus can be identified by the shape and color of the colony.

Clinical manifestations, especially in the initial stages of the disease, are uninformative, and therefore interviewing the patient allows one to avoid an erroneous diagnosis. Laboratory tests allow one to suspect pemphigus, as acantholytic cells are found in fingerprint smears during cytological examination.

At histological examination intraepidermal location of blisters is detected.

A dermatologist can cure pemphigus. However, depending on the course of the disease, consultation with additional specialists may be necessary.

Often this is a surgeon or infectious disease specialist. For a complete diagnosis, only an examination by a dermatologist is sufficient.

But at the same time, the specialist excludes everything possible diseases, according to clinical manifestation diseases.

For a detailed diagnosis of a disease in a child, you may need:

General blood analysis.
Histological analysis of the contents of the vesicles.
Immunological research.
Carrying out an antibiogram.

If acantholytic pemphigus is suspected, a cytological examination of a smear from the ulcer is performed. During the examination, the laboratory technician can detect acantholytic epidermal Tzanck cells, which indicate the presence of pemphigus in the patient.

A histological examination of the skin area can also be performed. With pemphigus, intercellular edema in the epidermis, acantholytic disturbances of integrity, and blisters are detected.

By using immunological research Between the epidermal cells in the area of the bladder, deposits of IgG and IgA can be detected.

Visual inspection. Most often, experienced dermatologists make an accurate diagnosis by detecting the patient’s symptoms, which we described above.
Smear-imprint. It is needed for detection acantholytic cells, which are characteristic of all forms of pemphigus.
Histological examination. Thanks to it, a specialist can detect cracks, blisters, and degeneration of cells of the granular layer between the layers of the epidermis.
Immunological studies. They are needed for confirmation autoimmune mechanism development of the disease. Direct/indirect RIF are not always effective, they provide an opportunity to distinguish vulgar, leaf-shaped pathology.

Differential

In addition to these studies, it will also be necessary differential diagnosis the dermal lesion in question from:

Treatment

Dangerous complications of pemphigus are meningitis and encephalopathy - damage to brain cells and (or) its membranes, leading to human death.

The following therapy is carried out:

Antiviral drugs: Cycloferon (350 rubles), Lavomax (730 rubles), Acyclovir (25 rubles).
Anti-inflammatory and painkillers: Nimesulide (100 rubles), Ibuprofen (40 rubles).
Antihistamines: Loratadine (20 rubles), Zodak (125 rubles).
Disinfecting solutions: Miramistin (230 rubles), Chlorhexidine bigluconate (12 rubles).
External ointments: Acyclovir (20 r.), Solcoseryl (250 r.).

The course of treatment for pemphigus takes about 2 months, but the likelihood of relapse remains. After completion of therapy, the patient is recommended to strengthen his immune system.

To strengthen it and prevent the disease, smoked and fatty foods should be excluded from the diet. Walking on walks is remarkably helpful in reducing the risk of relapse fresh air and physical education classes.

A hypoallergenic diet and exclusion from the diet of roughage, canned food, simple carbohydrates, salty foods and other extractive substances are indicated for patients with any form of pemphigus. If the oral cavity is affected, then it is necessary to include puree soups and mucous porridges in the diet in order to prevent a complete refusal of food; protein-rich foods included in the diet accelerate the process of cell regeneration and epithelization of open erosions.

All patients with pemphigus should be on dispensary observation a dermatologist, a gentle regimen is recommended, no physical activity and avoiding sun exposure. Frequent changes of underwear and bed linen prevent secondary infections.

It is indicated to prescribe glucocorticosteroids immediately in high doses, as otherwise therapeutic effect will not be achieved after cupping acute manifestations pemphigus, the dosage of hormonal drugs is gradually reduced to the minimum effective. In the treatment of pemphigus, methods of extracorporeal hemocorrection are used: hemosorption, cryoapheresis and membrane plasmapheresis. As a local treatment for pemphigus, aniline dyes and non-aggressive antiseptic solutions are used.

Acute forms of pemphigus are often treated with corticosteroid hormones.

Very often doctors prescribe drugs:

prednisolone;
polcortolon;
metipred;
dexamethasone.

Contraindications to the use of hormones are peptic ulcers of the stomach or duodenum. In cases where such indications are present, hormonal drugs are administered intramuscularly.

The treatment process for pemphigus is quite complicated. Therefore, self-medication of this type of disease is under no circumstances acceptable. The disease progresses rapidly, affecting large areas of the skin, which leads to disruption of the internal organs.

Treatment of pemphigus in mandatory carried out in a dermatological hospital. First of all, corticosteroid drugs, cytostatics and other drugs are prescribed to alleviate the course of the disease and the life expectancy of patients.

The drugs must first be taken in large doses. At the same time, pay attention to blood and urine sugar levels, monitor blood pressure and observe personal hygiene rules.

With frequent changes of bed linen and underwear, secondary infection is prevented.

Patients with pemphigus are prescribed corticosteroid drugs in combination with anabolic steroid hormones, as well as calcium, potassium and ascorbic acid.

The drugs are taken for a long time until complete absence skin rashes. In no case should corticosteroid therapy be canceled or stopped, in case of improvement, as this will lead to an exacerbation of the disease.

Patients must be followed up with a dermatologist. Outpatients are advised to reduce physical activity and avoid nervous stress, be sure to follow a sleep schedule. During treatment, it is advisable not to change climatic conditions.

When treating pemphigus, the attending physician prescribes hormones (Prednisolone) internally, as well as externally (Betamethasone). Moreover, contraindications are relegated to the background. This is a distinctive feature of pemphigus disease.

Reduce daily dose Prednisolone should be taken very carefully and gradually. When there is a noticeable improvement in the patient's condition, the daily dose of Prednisolone is reduced every 4-5 days by 2.5-5 mg.

Thus, a maintenance minimum dose of the drug will be achieved, which will ensure remission of the disease.

The use of hormones in combination with cytostatic drugs, immunosuppressive (Sandimmune) leads to the cure of the patient in more short time and when using smaller daily doses of drugs. This achieves positive result in the treatment of pemphigus.

Sandimmune is first prescribed in half the dose to establish patient tolerability of the drug. Then the daily dose is divided into 2 doses with an interval of 12 hours. With a clear improvement in the patient's condition and a decrease in ulcerations, the daily dose of the drug is reduced.

After complete cleansing of the skin, the disease is not considered defeated; the patient must still take a minimum dose of Sandimmune, selected individually for each person, even after achieving remission. As maintenance therapy, the drug should be taken for 2-4 months.

In combination with hormones, Azathioprine is used in 2-4 doses per day and Methotrexate – once a week.

When treating pemphigus, blood purification methods are also used - hemosorption and plasmapheresis.

Hemosorption is used in patients with concomitant severe diseases such as atherosclerosis, diabetes, hyperthyroidism. During hemosorption, immunoglobulins and other pathogenic components are removed.

Plasma transfusion during plasmapheresis is carried out at intervals of 7 to 14 days. In one procedure, from 500 to 2000 ml of plasma is removed with the introduction of donor plasma or plasma substitute. As a result, circulating immune complexes are removed from the blood of patients.

The essence of the photochemotherapy method in the treatment of pemphigus comes down to the inactivation of blood cells using G-methoxypsoralen in combination with irradiation of the cells with ultraviolet rays and their introduction into the blood. As a result of these procedures, the blood is cleared of toxic substances, as well as immunoglobulins.

This disease must be treated promptly. Therapy should be carried out under the supervision of a dermatologist after making an accurate diagnosis. Treatment is carried out exclusively with medication.

In a therapeutic way

As a complement to drug treatment you can use a therapeutic method. Dermatologists can recommend local baths that contain disinfectants, anti-inflammatory agents, and astringent additives. So, you can use a decoction made from oak bark for baths.

By medication

Treatment is aimed at reducing the body's production of antibodies to its own tissues. Basic medicines, used in the therapy of this dangerous disease, these are corticosteroid hormones.

Most often, dermatologists prefer prednisolone. Dermatologist in individually selects a treatment regimen. To start, a dose is selected that is equal to 80–100 mg of the drug per day. It is necessary to take 2/3 of the prescribed daily dose in the morning.

A noticeable effect from hormone therapy appears by 10–14 days of using the medication. From this time on, the dose of the drug used should be reduced. The first reduction is usually 25–30%, after which the dosage reduction is introduced more gradually.

Glucocorticosteroids may be prescribed:

"Triamcinolone".
"Prednisolone."
"Dexamethasone".
"Methylprednisolone."

If the case is very severe, the dermatologist prescribes immunosuppressants:

The effect in treating the disease was noted with the simultaneous use of corticosteroids and synthetic antimalarials (Hydroxychloroquine, Chloroquine).

The folk way

Different variants traditional medicine can be used as an addition to the therapy prescribed by the dermatologist. To treat the affected areas you can use:

puffball mushroom (its pulp);
nettle leaves (juice squeezed from them);
oil tincture made from fresh leaves walnut.

Treatment for pemphigus begins after diagnosis. This is done based on an examination and conversation with the patient’s parents or the patient himself, if he is an adult.

The diagnosis is performed either by an infectious disease specialist or a dermatologist (a joint examination of these two specialists is more often used). After the examination, it is necessary to donate blood from a vein to check for antibodies to the enterovirus, but treatment is prescribed immediately, since the diagnosis of the virus will last at least 2 weeks.

Therapy is as follows:

If the blisters are itchy, prescribe antihistamines: “Fenistil”, “Erius”, “Zodak”, “Suprastin”. In case of severe itching, 2 of these drugs (for example, Suprastin and Erius) can be combined without exceeding the daily dosage.
In case of severe itching of the rash, general treatment is supplemented by treating the blisters with local antihistamines: “Psilo-balm”, “Fenistil-gel”.
To relieve the pain of the rash and reduce body temperature, Nurofen, Paracetamol, and Nise are used (the latter only for adults). "Aspirin" or acetylsalicylic acid It is unacceptable for children to use!
Apply a special diet: exclude from your diet hot, smoked, sour and spicy foods and drinks that will irritate the inflamed oral mucosa. Also exclude hot food, give preference to those dishes and drinks that feel more harmonious when cold (okroshka, compotes, ice cream, fruit ice).
Rinse your mouth with antiseptic solutions: aqueous solution furacillin, chlorhexidine. For adults, you can use Orasept, Strepsils spray with lidocaine and other sprays containing an antiseptic and anesthetic.
Blisters on the skin can be treated with fucorcin or a solution of brilliant green.

In some cases, infectious disease doctors prescribe antiviral drugs. For children this is “Viferon” or “Laferon” in suppositories, for adults - “Cycloferon” in tablets or “Laferon” in the form of intramuscular injections.

To relieve itching, in addition to official medicine, folk remedies are successfully used:

The main treatment for this disease is taking corticosteroid hormonal drugs, such as prednisolone. The dosage is 80-100 mg/day to relieve the disease and 200 mg/day to treat advanced cases.

The effect of taking the medication will be noticeable within two weeks after starting treatment. Then the dose is reduced to 5 mg/day to avoid relapse. In addition to prednisolone, urbazone, triamcinolone or metypred are also used.

Hormone therapy often causes complications, such as obesity, diabetes, gastric ulcers, hypertension, thromboembolism, pancreatitis and decreased immunity. Therefore, in order to avoid the appearance concomitant diseases should be observed special diet, rich in vitamins and protein, limit salt and carbohydrate intake.

Potassium chloride (3 g/day) and, if necessary, antibiotics are also prescribed.

In addition, they apply medicinal baths with a weak solution of potassium permanganate or oak bark, external use of ointments and brilliant green, various oils to soften the skin.

Painkillers such as novocaine and disinfectants such as hydrogen peroxide are used.

Patients must be under the supervision of a doctor and undergo blood and urine tests once a month. It is necessary to avoid physical activity and adhere to the regime. Climate change and self-medication are not advisable.

The only effective way to treat this disease is through the use of medications. As an auxiliary method, you can use a therapeutic one.

Therapeutic

Along with the use of medications, extracorporeal hemocorrection is prescribed. To purify blood, the following are most often used:

Medication

Drug therapy involves the use of the following groups of drugs:

Therapy for pemphigus of any etiology always begins with taking loading doses of hormones such as Prednisolone, Dexamethasone and the like. The dosage is determined by the attending physician individually, calculating it based on the severity of the disease.

Treatment with corticosteroids is very long-term and can last for several months. The patient takes loading dose until the formed blisters and erosions begin to heal and disappear.

After which the amount of the drug is slowly reduced to a certain minimum.

Folk remedies for pemphigus

Thanks to alternative medicine it is impossible to get rid of this dermatological disease like pemphigus, but it really relieves painful rashes. The following recipes will help reduce inflammation and speed up the healing process of formed wounds:

soak napkins with juice from fresh nettle leaves and apply to the erosion or wound. This compress has a wound-healing, hemostatic and analgesic effect;
the same can be done from the juice of green leaves tree aloe, the effect will be identical;
mix onion, garlic, salt, pepper and honey in equal proportions and simmer in the oven for at least 15 minutes. Cool the resulting viscous slurry and lubricate the opened bubbles. In addition to healing wounds, the product helps to draw out purulent contents;
2 tablespoons chopped flower heads red clover pour one glass of boiling water and leave for about 2 hours. After which the decoction can be used to wash the resulting erosions due to pemphigus, which will facilitate their speedy healing.

As a preventive measure against pemphigus, the simplest recommendations are effective: wash your hands as often as possible - after using the toilet, visiting public places, streets; monitor your immunity, avoid overwork; respond promptly to signs of any disease and do not neglect full course treatment; stick to healthy eating And active image life.

Complications

Enteroviral pemphigus is a disease that is usually mild, but in the presence of a weakened immune system it can be complicated:

meningitis - inflammation of the membranes of the brain. In most cases it has mild course, ending with recovery;
encephalitis - inflammation of the brain. Rarely develops, can occur in the form varying degrees heaviness;
pneumonia;
myocarditis – inflammation of the heart muscle, which without proper treatment can result in heart failure. The cause of myocarditis is that the sequence of antigens that myocardial cells display to the immune system (as almost all cells do) is in a particular region identical to those of the Coxsackie virus, which causes viral pemphigus. The immune system “thinks” that the myocardium is a microbe and begins to attack it.

Developing in the first three months of pregnancy, viral pemphigus can cause miscarriage. Under the influence of this virus, severe fetal malformations can form, due to which artificial premature birth will have to be induced.

Due to the large number of side effects of glucocorticoids, serious complications are possible. Long-term use of these drugs may cause:

Prevention

The best way to avoid pemphigus in a child is to follow preventive recommendations.

The main preventive measures for pemphigus are:

Follow the doctor's instructions.
Do not interrupt treatment with hormonal drugs.
Eliminate exposure to provoking factors.

Pemphigus in children of any age requires mandatory and precise implementation drug therapy. As well as correction of the child’s nutrition and lifestyle.

The essence of prevention is to maintain a healthy lifestyle. The patient is recommended:

good nutrition;
healthy sleep;
walks in the fresh air (it is recommended to avoid the sun).

There are no vaccines or serums for enterovirus - there are so many strains that it is impossible to guess which one you will come into contact with. If you or your child has been in contact with someone who has pemphigus, to reduce the chance that you will get sick, you need to eat well for the next week, enriching your diet sufficient quantity vitamin products(these are fruits, vegetables, natural freshly squeezed juices, raisins).

It is also worth consulting with your doctor about whether you can take calcium supplements, and, if this does not harm your health, drink Calcium-D3 or Calcium gluconate in an age-appropriate dosage for 3-7 days.

If there is a history of pemphigus, it is necessary to take maintenance therapy in the form of hormones. Healthy people need to monitor blood and urine sugar levels and maintain normal blood pressure.

To prevent viral pemphigus, you should wash your hands often with soap and practice personal hygiene.

After eliminating the signs of the disease, you should think about a number of preventive measures that are necessary to prevent relapses. They are:

monitoring the condition of the dermis;
taking vitamins, calcium, potassium;
monitoring the occurrence of adverse reactions after taking medications;
control (regular) sugar levels in urine and blood;

Pemphigus is a chronic disease associated with disruption of the immune system. It appears as bubbles different sizes on apparently healthy skin and mucous membranes. According to their clinical characteristics, four forms of the disease are distinguished - true (vulgar), leaf-shaped, erythematous and vegetative. The diagnosis is confirmed if acantholytic cells are detected during a histological examination of a fingerprint smear taken from damaged skin. For treatment, general and local action containing glucocorticosteroids in combination with one of the methods of extracorporeal blood correction (hemocorrection) - criapheresis, hemosorption, plasmaphoresis.

Features of pemphigus in adults

Depending on the clinical picture pathological process, there are two main types of pemphigus - true (acantholytic) and benign (non-acantholytic). The first is more severe and dangerous, manifests itself in different forms, and if left untreated, leads to complications that are dangerous to health and life. The second is easier and milder, it rarely causes complications, manifests itself in different forms, but is not so dangerous to health and life.

Acantholytic forms:

Brazilian.
Vegetative.
Vulgar (ordinary).
Leaf-shaped.
Erythematous.

Non-acantholytic forms:

Scarring non-acantholytic.
Nonacantholytic.
Bullous.

Rare forms include pemphigus:

Herpetiformis. Its symptoms are similar to Dühring's dermatitis and are accompanied by a rash in the form of erythematous plaques and small superficial blisters with clear liquid inside. IN acute form the signs are the same as for the erymatous and ordinary forms.
Medicinal. The causes of the disease are genetic predisposition, intake of certain medicines against the background of decreased immunity. In the first case, the signs disappear after long-term treatment, in the second - almost immediately after stopping the medications. Most often these are Pyritinol, Tipronin, Penicillamine, Bucillamine, Piroxicam and preparations containing gold. Symptoms are the same as for pemphigus vulgaris, foliaceus or erythematous pemphigus.
Paraneoplastic. Develops against the background of malignant tumors, is life-threatening, in 90% of cases leads to fatal outcome. Most often diagnosed with hematological types of cancer - lymphocytic leukemia, macroglobulinemia, lymphomas.
IgA. There are two types of this variety - subcorneal pustular dermatosis and intraepidermal neutrophilic IgA dermatosis. Only a doctor can determine this or that variety. laboratory tests and clinical picture. What does IgA pemphigus and its other forms look like? different stages, demonstrate the photos below.

Symptoms of pemphigus in adults

Some symptoms of pemphigus are common to all types and forms. This is rapid progress in the absence of treatment and wave-like behavior - the disease either subsides or intensifies again. Other signs depend on the type and form of the disease:

Vulgar (ordinary) pemphigus. Bubbles of varying sizes spread throughout the body. They have a flaccid and thin cover (surface), inside they are filled with a transparent or translucent liquid - serous exudate. Most often, the first bubbles appear on the mucous membranes of the mouth and nose, which causes:

Pain when chewing, swallowing and talking.
Increased salivation.
Pain when blowing nose.
Bad breath.

The pathological process on the mucous membranes drags on for a long time - from 3 months to 1 year. Then blisters appear on different areas bodies. Often this happens so quickly that the patient does not notice their formation. Sometimes the blisters burst, and in their place, bright pink erosions with a smooth, glossy surface form, causing pain, then dry crusts form. As a rule, they spread from the center of the cluster of bubbles to the edges and form large zones. When diagnosing pemphigus vulgaris, the Nikolsky test gives a positive result - with a slight impact on the skin in the affected area, the top layer peels off. During illness, weakness is felt and the temperature rises.

Erythematous pemphigus. First, blisters appear on the chest, neck, face and scalp, the symptoms are similar to seborrhea - clear boundaries of affected areas, rapid self-opening of blisters with a sluggish and flabby surface, erosion, brown or yellowish crusts of varying thickness in their place. Nikolsky syndrome is local in nature, but gradually affects other parts of the body.

Pemphigus vegetans. Benign disease, which lasts for many years without worsening the well-being of patients. Bubbles first appear in the natural folds of the skin, around the mouth, nose and ears, in the genital area and anus. They open spontaneously, and in their place erosions form with an unpleasant-smelling serous-purulent or serous coating, surrounded by pustules. When making a diagnosis, the disease must be differentiated from chronic vegetative pyoderma; the Nikolsky test gives a positive result only in the affected areas.

Pemphigus foliaceus. First, flat blisters, slightly raised above the skin, appear on the body, then form on the mucous membranes. Characteristic sign– simultaneous presence of bubbles and crusts, layered on top of each other.

Brazilian pemphigus. It is found only in Latin American countries (Venezuela, Paraguay, Peru, Bolivia, Argentina and Brazil, etc.), and has never been detected on other continents. The cause of the occurrence has not yet been established, but most likely the disease has infectious nature. More often, Brazilian pemphigus is diagnosed in women under 30 years of age and affects only the skin. First, flat blisters appear, then they open and become covered with scaly exfoliating crusts, under which erosions form that do not heal for several years. Nikolsky syndrome in the affected areas gives a positive result, the disease causes severe discomfort - accompanied by pain and burning.

Bullous pemphigus. It proceeds benignly and is not accompanied by acantholysis (destruction of the skin). Blisters appear on different parts of the body, which disappear without a trace as a result of treatment or on their own.

Nonacantholytic pemphigus. It proceeds benignly, manifests itself as bubbles on the oral mucosa, there are signs of inflammation, and ulceration of the affected areas is observed.

Scarring non-acantholytic. Blisters form on the mucous membranes of the eyes and mouth. The risk group includes women over 45 years of age. This form of the disease has another name – pemphigus of the eyes.

Causes of pemphigus in adults

Most often, the cause of pemphigus vulgaris is changes in tissue and skin cells, as a result of which they become antibodies for the immune system. Such metamorphoses occur when exposed to aggressive environmental factors or retroviruses. Changes in epidermal cells and the synthesis of specific antigens disrupt intercellular communication, resulting in the formation of specific blisters on the surface of the skin. Other provoking factors have not been identified, but it is known that the incidence rate is influenced by genetic predisposition.

Pemphigus in children

As a rule, pemphigus in children is diagnosed in the first months of life. It is highly contagious (contagious) infection, manifested in the form of pustules spreading very quickly over the skin. Pemphigus in children is bacterial in nature; the causative agent of the disease is Staphylococcus aureus.

Due to the reactive characteristics of the skin, which are aggravated by the unhealthy lifestyle of pregnant women, premature birth and birth injuries, children are practically not protected from bacterial infections. As a result, blisters with serous contents may appear on the skin already in the first days of life. The disease can manifest itself 1-2 weeks after birth. There are other provoking factors:

Violation of hygiene rules in maternity hospitals.
Maternity hospital staff as carriers of infection.
Purulent inflammation of the navel.

Pemphigus develops very quickly in children. The blisters almost instantly spread throughout the body and increase in size, bursting after a few hours. In their place, erosions form with remnants of skin along the edges, which cause pain and become covered with purulent crusts. The process is accompanied by intoxication, elevated temperature, lack of appetite.

Diagnosis of pemphigus in adults

The disease is diagnosed by visual examination; it is differentiated from syphilitic pemphigus, which is a consequence congenital syphilis with localization of blisters on the palms. In some cases it is required additional research on the:

Tzanck cells (cytological).
Intraepidermal blisters (histological).
Suprabasal glow (immunofluorescent).

With classical development, the diagnosis of pemphigus is not difficult. In addition to congenital syphilis, it must be differentiated from lupus erythematosus (bullous form), congenital epidermolysis bullosa, bullous toxicoderma, and exudative erythema multiforme.

Treatment of pemphigus in adults

Since scientists still cannot establish exact reasons, treatment of pemphigus in adults causes some difficulties. Patients are placed on dispensary registration, they should avoid excessive physical activity and stress, change clothes and bedding as often as possible, follow a diet and hygiene rules.

The basis of treatment is drug therapy:

High doses of glucocorticoids (Polcortolone, Metipred, Dexamethasone, Prednisolone). If regression of the disease is observed, the dosage is gradually reduced.
For gastrointestinal diseases, long-acting glucocorticoids are prescribed - Diprospan, Metipred-Depo, Depo-Medrol.
Treatment is supplemented with hormonal drugs, the disadvantage of which is a high probability of complications: depression, insomnia, arterial hypertension, increased excitability, angiopathy, weight gain, thrombosis, ulcers (erosions) of the stomach (intestines), steroid diabetes.
If the condition worsens, medications are prescribed to restore the gastric mucosa (Almagel, etc.), a diet that includes a minimum of fats and carbohydrates, a maximum of protein and vitamins.
In parallel, immunosuppressants and cytostatics are taken - Azathioprine, Methotrexate, Sandimmune.
To avoid violation electrolyte balance, it is recommended to take potassium and calcium.

Additional treatments:

Blood purification - hemodialysis, plasmapheresis, hemosorption. Such procedures remove immunoglobulins, toxic substances and circulating immune complexes from the blood. They are especially useful for patients with hyperthyroidism, atherosclerosis, and diabetes.
Photochemotherapy - effects ultraviolet rays in parallel with G-methoxypsoralen, it reduces the activity of blood cells and directs it into the blood vessels, cleanses the body of toxins and immunoglobulins.
Local treatment - ointments with glucocorticoids, sprays with xylocaine, lidocaine and others local anesthetics, solutions with aniline dyes (brilliant green, Fukortsin), baths with potassium permanganate, treatment of ulcers with Kuriozon.
Diet - exclude from the diet foods that can cause allergies, rough foods, simple carbohydrates, salt, canned food. The menu includes products with high content protein and vitamins. If blisters form in the oral cavity, puree soups and soft porridges are recommended, excluding mechanical damage mucous membrane.

Complications of pemphigus in adults

If left untreated, pemphigus provokes inflammation of internal organs, pneumonia, phlegmon, and otitis. In newborns, severe septic form of the disease can be fatal. In adults, there is a high probability of secondary infection. Pemphigus vulgaris can cause damage to the kidneys, liver, of cardio-vascular system, leaf-shaped - sepsis and death.

Prevention of pemphigus in adults

The main measures to prevent pemphigus are regular changes of underwear and bed linen, compliance with hygiene rules, healthy image life and proper nutrition.

Pemphigus is a chronic autoimmune disease characterized by special type bubbles on the surface earlier healthy skin and mucous membrane. Among the types of pemphigus can be distinguished: vulgar, vegetative, erythematous and foliate.

Pemphigus can be diagnosed if acantholytic cells are detected, which are detected in a smear taken or as part of blisters in the epidermis itself (during histological examination). To treat pemphigus, glucocorticosteroids are first used (a whole course of treatment is prescribed). The latter always goes well with extracorporeal hemocorrection (plasmophoresis, cryoapherosis, hemosorption).

What it is?

Pemphigus is a serious disease that affects the human skin. As a result of its progression, pathological blisters are formed on the skin and mucous membranes, filled with exudate inside. This process begins due to the stratification of the epithelium. Pathological foci can merge and tend to grow rapidly.

Causes

The reasons for the development of pemphigus have not yet been fully studied. One of the main causes of pemphigus is a violation of autoimmune processes, thereby the cells become antibodies to the immune system.

Violation of cell structure is subject to the influence of external factors, as well as aggressive environmental conditions. As a result, the communication between cells is disrupted, which leads to the formation of bubbles. The incidence rate in people with a hereditary predisposition is much higher.

Mechanism of bubble formation

The epidermal layer consists of 10-20 cell layers, which look like bricks under a microscope. The “bricks” of the second layer of the epidermis are connected to each other by peculiar “bridges”. On top of the “wall” there are layers of cells that are no longer quite similar to cells, reminiscent of applied cream. These are scales, corneocytes, necessary for protection from mechanical, chemical and physical damage.

If, under the influence of internal or external causes, antibodies are formed that destroy the “bridges” - desmosomes between the cells of the basal layer (this is called acantholysis and can be seen under a microscope), this is true pemphigus. If tissue fluid penetrates between the basal and upper layers of the epidermis without destroying the “bridges,” it is pemphigoid. Viral pemphigus also occurs without destruction of desmosomes.

Classification

Types of non-acantholytic pemphigus:

Non-acantholytic pemphigus is benign. Pathological elements are formed exclusively in the human oral cavity. Upon examination, inflammation of the mucous membrane, as well as its slight ulceration, can be detected.
Bullous form of non-acantholytic pemphigus. This is a benign disease that develops in both adults and children. Blisters form on the skin, but there are no signs of acantholysis. These pathological elements can spontaneously disappear without scarring.
Cicatricial non-acantholytic pemphigus. This pemphigoid is called pemphigus of the eye in the medical literature. Most often it is diagnosed in women who have crossed the 45-year age limit. Characteristic symptom– damage to the visual apparatus, skin and oral mucosa.

Classification of true pemphigus:

Erythematous form. The pathological process combines several diseases. Its symptoms are similar to seborrheic dermatitis, an erythematous variant of systemic lupus, as well as true pemphigus. Erythematous pemphigus in adults and children is very difficult to treat. It is worth noting that the disease is diagnosed not only in people, but also in some animals. A characteristic symptom is the appearance of red spots on the skin of the body and face, covered with crusts on top. Simultaneously with this symptom, seborrheic manifestations appear on the scalp.
Pemphigus vulgare. This type of pathology is diagnosed in patients more often. Blisters form on the skin, but there are no signs of inflammation. If pemphigus is not treated on time, pathological elements can spread throughout the entire skin. It is worth noting that they can merge and form large lesions.
Pemphigus foliaceus. This form received its name due to the characteristics of the pathological elements. Blisters form on the human skin, which practically do not rise above the epidermis (not tense). Crusts form on top of them, which tend to layer on top of each other. The effect of sheet material folded in stacks is created.
Brazilian pemphigus. There are no restrictions regarding gender and age. Cases of its development have also been recorded in children early age, and in older people aged 70 to 80 years. It is also possible that it may progress in middle-aged people. It is worth noting that this variety is endemic and is therefore found only in Brazil.

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Symptoms

Considering that experts have identified several various types given pathology, then the symptoms for each of them will be very specific. Of course, there are a number of general trends and signs inherent in all types of the disease. This may include, for example, the wave-like course of the pathological process.

Periods of exacerbation alternate with the transition of pemphigus to a calmer stage, when the main symptoms subside or completely disappear. An important factor for the patient will be the fact that in the absence timely diagnosis and prescribing an effective course of treatment, there is a high risk of developing severe conditions aggravated by concomitant diseases.

The presence of crusts, ranging from pale pink soft to red dense, reminiscent of lichen;
There is a deterioration in the general condition;
Decreased immune response of the body;
Formation of bubbles of varying densities;
Also, in severe cases, separation of the layers of the epidermis is noted, and it can occur both in the lesion and away from it.
Damage and ulcers of the mucous membrane of the mouth, nasopharynx or genitals;
Pain when performing the act of swallowing or when eating;
Bad breath, indicating damage to the mucous membranes;
Hypersalivation or, in other words, increased salivation;
With seborrheic form on hairy skin The heads develop characteristic crusts of a yellowish or brownish-brown color.
Bubbles of various appearance, ranging from flat to thin-walled, which burst with a slight touch. In their place, erosions and, subsequently, crusts form.
In severe cases, an eroded surface of the skin may form in place of the blisters. Their feature is a tendency towards peripheral growth. Over time, such erosions occupy a large surface of the skin, causing pain and inconvenience to the patient.
In children, manifestations of pemphigus are localized over the entire surface of the skin, including the limbs.

Experts say that with this disease, both a pure form of the pathological process and mixed forms that smoothly transform into one another can be observed. Therefore, the symptoms and signs of pemphigus in a given person may vary and indicate the presence of several types of disease.

What does pemphigus look like: photo

The photo below shows how the disease manifests itself in humans.

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Diagnostics

Experts say that a correct diagnosis can be made based on a comprehensive examination of the patient, which includes several important stages:

Examination of the patient for the presence of a clinical picture. At this point, the doctor establishes the nature of the lesions, their localization, the degree of development of the disease, etc.
Cytological analysis necessary to establish the presence of acantholic cells in smears of biomaterial.
Carrying out the Nikolsky test, which allows to differentiate pemphigus from similar pathological processes.
Method of direct immunofluorescence. This study allows you to detect the presence of immunoglobulin in the intercellular substance of the epidermis.
A histological study, which is based on a technique for detecting crevices and other damage within the epidermis.

Only the totality of all results makes it possible to make an accurate diagnosis and prescribe effective course treatment leading to the patient's recovery.

Treatment of viral pemphigus

Treatment of viral pemphigus involves the use of the following systemic drugs:

cytostatics stop the division of immune cells: Sandimmune, Azathioprine, Methotrexate;
antiviral: Viferon, Laferon, Cycloferon;
glucocorticosteroids: Dexamethasone, Prednisolone;
antipyretics: Ibuprofen, Paracetamol, Nimesil, Mefenamic acid;
antihistamines relieve itching: Cetrin, Diazolin, Fenistil.

For external treatment of affected skin areas, the following may be prescribed:

antimicrobial local anesthetics for irrigating the oral cavity if viral pemphigus has affected the child’s mucous membranes: Forteza, Orasept;
antiseptics: Chlorhexidine, Methylene blue, Miramistin;
combination preparations of antiseptics and anesthetics: Oflokain, pharmaceutical talkers;
antipruritic lotions made from nettle juice, aloe, and walnut oil.

Since children with this diagnosis are usually treated in inpatient conditions, to enhance the therapeutic course can be carried out healing procedures aimed at purifying the blood of antibodies:

plasmapheresis - replacement of the liquid part of the blood with similar solutions without microbes, immune complexes and antibodies;
hemosorption using a carbon filter.

Only a doctor can tell how to treat viral pemphigus, because in each individual case it can acquire some special features. As for other forms of pemphigus, the therapeutic course for them is also determined individually.

How to treat other forms of pemphigus?

Treatment of pemphigus is mandatory in a dermatological hospital. First of all, corticosteroid drugs, cytostatics and other drugs are prescribed to alleviate the course of the disease and the life expectancy of patients.

The drugs must first be taken in large doses. At the same time, pay attention to blood and urine sugar levels, monitor blood pressure and observe personal hygiene rules. With frequent changes of bed linen and underwear, secondary infection is prevented.

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Medicines for the treatment of pemphigus

The patient is advised to take glucocorticoids in high doses. The following drugs can be used for this:

Metipred;
Prednisolone;
Dexamethasone;
Polcortolon.

When symptoms begin to regress, the doses of these drugs are gradually reduced to the minimum effective. Patients with pathologies of the gastrointestinal tract are prescribed long-acting glucocorticoids:

Metipred-depot;
Diprospan;
Depo-Medrol.

Treatment with hormonal drugs can cause a number of complications, but they are not a reason to discontinue corticosteroids. This is explained by the fact that refusal to take them can lead to relapses and progression of pemphigus.

Possible complications during treatment:

acute psychosis;
arterial hypertension;
depressive states;
insomnia;
increased excitability of the nervous system;
steroid diabetes;
thrombosis;
obesity;
angiopathy;
erosions or ulcers of the stomach and/or intestines.

At sharp deterioration condition of the patient while taking corticosteroids, the following measures may be recommended:

diet: limiting fats, carbohydrates and table salt, introduction to diet more protein and vitamins;
drugs to protect the gastric mucosa: Almagel, etc.

In parallel with glucocorticoids to increase the effectiveness of therapy and the possibility of dose reduction hormonal drugs cytostatics and immunosuppressants are prescribed.

The following medications can be used for this:

Sandimmune;
Methotrexate;
Azathioprine.

To prevent electrolyte imbalance, the patient is recommended to take calcium and potassium supplements. And for secondary infection of erosions - antibiotics or antifungal agents.

The ultimate goal of drug therapy is to make the rash disappear.

Preventive measures

There are no specific measures to prevent the development of pathology. The higher the level of immune protection, the less chance of dermatological diseases.

control the nature of chronic diseases;
strengthen immunity;
maintain personal hygiene;
Healthy food.

Measures to prevent pemphigus in newborns:

change your underwear more often;
Caring for newborns with pustular skin lesions is prohibited;
Take regular care of your child’s skin;
strengthen immune system weakened children;
needed daily wet cleaning, room ventilation.

If you notice any rashes on the skin, the formation of pustules and blisters, immediately contact a dermatologist.

Forecast

The prognosis for acantholytic pemphigus is conditionally unfavorable. On the one hand, in the absence of effective treatment, there is a high probability of complications and death.

On the other hand, patients with pemphigus are forced to take glucocorticosteroids for a long time, and sometimes for life, which is fraught with the development of side effects. But hasty refusal of drugs leads to immediate relapse of the disease. Glucocorticosteroids do not eliminate the cause of the disease, but inhibit the pathological process and prevent its progression.