Pemphigus vulgaris: causes, symptoms, treatment, drugs. Autoimmune diseases

Departments - Skin and venereal diseases. Clinical diagnosis: Main: idiopathic widespread eczema. Concomitant: chronic cholecystitis, chronic pancreatitis, duodenal ulcer, stage III hypertension, angina pectoris. Teacher: E. Vvedenskaya. Year: 2.

Pemphigus vulgaris (VP) is a severe autoimmune disease with a steadily progressive course. In this regard, retrospective studies of 48 case histories of patients with pemphigus vulgaris for the period from 2001-2005 were carried out.

It is manifested by the formation of blisters on the skin and mucous membranes as a result of stratification of the epidermis. It occurs on both sexes, more often older than 4. Children get sick very rarely. The disease is severe and is manifested by the formation of blisters on non-inflamed skin and mucous membranes, which quickly spread throughout the skin.

In smears - prints obtained from the bottom of erosion, pathological cells can be detected. Without adequate treatment, the disease leads to death. According to the symptoms and course, 4 forms of the disease are distinguished.

Pemphigus vulgaris. On outwardly unchanged skin or mucous membranes, tense blisters the size of a pea, hazelnut or more appear, with a transparent, gradually cloudy content. Bubbles open, forming bright red erosions, or dry up, turning into a crust.

Persistent pigmentation remains at the site of the rash. The general condition of patients is severe, they suffer from insomnia, the temperature often rises, appetite worsens. Vegetative pemphigus. Quickly opening blisters are formed in the oral cavity, on the lips, in the armpits, inguinal folds, on the external genitalia.

Easily bleeding growths (vegetations) up to 1-2 cm high appear at the bottom of the erosion. In the stage of regression, the discharge shrinks into powerful loose crusts that cause excruciating pain. Leaf-shaped bladder. The sudden appearance on the face and trunk of flaccid blisters in the form of grouped foci is characteristic. Bubbles are easily destroyed, the exudate shrinks into lamellar thin crusts, similar to puff pastry. Very quickly, the pathological process spreads to the entire skin. The mucous membranes are usually not affected.

Radina, vulgar pemphigus often begins with a lesion of the larynx, which often remains unrecognized for a long time. Sometimes with such a course of the disease, spontaneous remission occurs, which can last weeks and months. Case history of pemphigus Disorders of the menstrual cycle 7. Diagnosis of pemphigus vulgaris In the clinical diagnosis of pemphigus vulgaris, mechanical symptoms are important, indicating acantholysis. Case history: Chron. DATE OF ADMISSION TO THE CLINIC: 27.04.2000 BY WHOM THE PATIENT IS REFERRED: polyclinic DIAGNOSIS AT ADMISSION: simple pemphigus CLINICAL DIAGNOSIS BASIC: Chronic. The medical history of this patient testifies to the lack of experience in the diagnosis of cystic dermatoses among the doctors who supervised him. Pemphigus vulgaris of the oral mucosa.

Seborrheic or erythematous pemphigus. It begins with the formation of small blisters on the face, scalp, back, chest, and sometimes the oral mucosa, which quickly shrink into crusts. Under them, an eroded surface is exposed. The disease proceeds for a long time and in most cases is benign.

Pemphigus treatment. The main means are corticosteroids and cytostatics.

Therapy should be given continuously indefinitely for a long time until the complete disappearance of the rashes. Antibiotics and sulfa drugs are prescribed in cases of secondary infection. Locally - general baths with potassium permanganate of low concentration, wheat bran extract, decoction of oak bark, antibacterial ointments or aqueous solutions of aniline dyes. The affected oral mucosa is irrigated with warm solutions of 0.2. Patients with pemphigus are on dispensary records and receive medicines free of charge during outpatient treatment. They need to avoid physical overload and nervous tension, observe the regime of rest and sleep. It is not allowed to change climatic conditions, treatment with mineral waters in resorts.

ANALYSIS OF ERRORS IN THE DIAGNOSTICS OF VULGARUS VULGAR.

Pemphigus vulgaris - causes, symptoms, diagnosis and treatment. Pemphigus vulgaris is the most common clinical form of pemphigus. The incidence of it in the world is 0.1-0.5 per 1. People usually get sick at the age of 3.

Like other forms of pemphigus, pemphigus vulgaris refers to bullous dermatoses, since its main element is a bladder. About 2/3 of cases of the disease begin with the appearance of blisters on the oral mucosa, and only after a few months the skin is involved in the process.

Causes of vulgar pemphigus. The development of pemphigus vulgaris is associated with disorders in the immune system, as a result of which the production of antibodies of the Ig type occurs. G to own cells of the spinous layer of the epidermis. Under the action of autoantibodies, the destruction of desmosomes that bind epidermal cells to each other occurs. The loss of connections between cells (acantholysis) leads to the fact that the space between them is filled with intercellular fluid with the formation of acantholytic blisters characteristic of pemphigus vulgaris.

Symptoms of pemphigus vulgaris. Most often, pemphigus vulgaris begins with the mucous membrane of the mouth and throat. Due to mechanical damage from food, the blisters burst so quickly that they are almost never seen. In the mouth, against the background of unchanged mucosa, bright red painful erosions are formed.

Fragments of a burst bubble covering erosion create a picture of a whitish coating on it, but they are easily removed with a spatula. Gradually, the number of erosion increases. Without specific therapy, they do not heal, but rather grow and merge. Due to severe pain, the patient cannot eat or talk. There is a fetid odor from the mouth.

The appearance of blisters on the skin with pemphigus vulgaris can occur several months after they appear in the oral cavity, but can also be observed at the onset of the disease. Blisters form on apparently unchanged skin, they are filled with a clear liquid and are often accompanied by neither itching nor pain. In some cases, reddening of the skin in the form of a thin rim is observed around the blisters. Pemphigus vulgaris is characterized by focal appearance of rashes in various parts of the body. The chest, back, armpits and inguinal folds are most often affected. Over time, new blisters appear with the involvement of previously healthy skin areas in the process. A few days after their appearance, the bubbles open.

The resulting erosions have a bright pink color. Gradually increasing in diameter, they merge and occupy vast areas of the skin. The general condition of the patient, not disturbed at first, with pemphigus vulgaris becomes worse, low-grade fever and weakness occur. Severe pain prevents active movements. When an infection is attached, pyoderma develops: the fluid in the blisters becomes cloudy, the erosions become covered with purulent discharge, and the patient's condition deteriorates sharply. Increasing cachexia or sepsis (with the addition of an infection) can lead to the death of the patient. Diagnosis of vulgar pemphigus.

In the clinical diagnosis of vulgar pemphigus, mechanical symptoms are important, indicating acantholysis. These include Nikolsky's symptom - exfoliation of the epidermis with light friction of a healthy-looking area of \u200b\u200bthe skin. The marginal symptom of Nikolsky is checked by sipping on a piece of the wall of a burst bladder. If it is positive, then there is a peeling of the epidermis at a fairly large distance from erosion. Asbo-Hansen's symptom - pressing a finger on the bladder with pemphigus vulgaris leads to exfoliation of the epidermis along the periphery of the bladder and an increase in its area. To confirm the diagnosis of pemphigus vulgaris, a cytological study is performed according to the Tzank method.

Microscopy of a smear-imprint obtained from the bottom of the erosion reveals characteristic acantholytic cells in the spinous layer of the epidermis. For histological examination, during a biopsy, a skin area containing a fresh bladder is taken.

Pemphigus vulgaris (this disease is otherwise called pemphigus vulgaris) is considered a disease with an autoimmune mechanism of development. Pemphigus is characterized by the fact that blisters appear on the mucous membrane, skin, which subsequently burst and form bright pink erosions in these places. It is very unpleasant that such affected places are connected, increasing, becoming quite extensive in area.

The cavities of the rash are filled with liquid and gradually disperse to other areas of the skin, subsequently bursting, forming weeping places of a pinkish color. Such erosive areas cause the evaporation of a decent amount of tissue fluid, which is so necessary for the body. In addition, these places are very favorable foci for the development of an additional infection.

In order to determine the cause of the formation of a bubble, a biopsy, histological and immunological examination of these neoplasms is performed. Treatment of pemphigus is carried out using corticosteroids, cytostatics, resort to the method of extracorporeal hemocorrection.

This disease in the chronic stage is very dangerous, therefore, it should be treated immediately, as soon as the first bubble appears and an appropriate diagnosis is made. Treatment is with immunosuppressive drugs. It is this treatment that allows patients to maintain the quality of their lives without serious complications during the course of the disease. The course of the disease is quite serious, the problem is that through the liquid (due to its loss) the body loses protein, in addition, erosion sites can fester.

The statistics of such illnesses

A fairly large group of diseases associated with a violation of the skin and the formation of blisters refers to vesicular dermatoses. Pemphigus vulgaris - a form of this group, occurs more often than others, accompanied by a rash on the skin or mucous membranes of the blisters. This disease occurs most often in women, if we talk about the “national predilections” of the disease, then this disease is more common in people of Jewish nationality, as well as in residents of East India and the Mediterranean. This fact is explained (according to scientists) by the presence of incestuous marriages among the designated peoples, as a result of which the structure of the gene is violated, which is responsible for the formation of the skin. According to the age characteristic, this is a disease of people from 30 to 60 years old.

The essence of the disease

The epidermis (upper layer of human skin) is characterized by a special structure. The epidermis consists of four layers, the palms and soles of the feet even have five layers. As part of the two lower layers - from fifteen or more layers. The cells in these layers are connected by special processes called desmosomes (they have a protein structure). Desmosomes are woven into cell membranes in a very strong network. Intercellular contacts even form a kind of "carabiners" that provide a strong and inextricable connection. This circumstance makes the skin durable, not tearing when rubbed or stretched.

On top of the germ cells, which are held together by desmosomes, there is an additional layer: granular. There are no intercellular contacts in the granular layer. Further on top there are two layers of dead cells. It is these layers of dead cells that protect the skin from various kinds of damage (mechanical, chemical, bacterial).

When the mechanism of the disease is triggered, the formation of antibodies occurs, with which the human immune system tries to destroy the desmosomes, and the result of this will be the separation of cells from each other. The described mechanism is called acantholysis, and the cells that have become isolated are Tzank cells. When a biopsy is performed, under a microscope, the result of these processes becomes apparent and an appropriate diagnosis is made.

When the cells that make up a whole layer of ten or even more layers are separated, fluid accumulates. As a result of complex processes (limitation of the interstitial fluid by cells with intact desmosomes), bubbles are formed - cavities up to five millimeters in diameter. The surface of the bubble is broken, the germ layer, already inflamed and therefore vulnerable to pathogens, is exposed. This layer becomes vulnerable to mechanical and chemical influences, which provokes bleeding and exposes the epidermal layer to infections. Since the liquid that has poured out of a burst bladder contains proteins, trace elements and electrolytes, their loss is detrimental to the body. And the more such bubbles, the more damage will be done to the body.

Causes

There is still no consensus on the causes of this disease. There are various versions and assumptions about the causes of the disease:

Endogenous - genetic, associated with immunity;
Exogenous factors - exposure to drugs: penicillin and its derivatives, interferons;
Physical factors (impact of harmful factors) - burns, ultraviolet radiation, radiation;
Viruses - herpes;
Diseases of the nervous system;
Violation of water-salt metabolism: a large amount of salts in the body and, as a result, fluid retention in the body;
Endocrine - this is mainly due to adrenal dysfunction;
Changes in the structure of enzymes that are responsible for the main biochemical reactions;
Food.

When summarizing all versions, you can group them into the following groups:

associated with the immune system;
Endocrine causes;
Causes of the nervous system;
Factors associated with improper metabolism;
exposure to infection;
Influence of toxic substances.

To date, medical scientists have not come to a consensus on the primacy or secondary nature of the changes characteristic of this disease. But most scientists agree on the infectious theory of the occurrence of pemphigus vulgaris. According to this version, a virus enters the body and remains there until a certain time not recognized. When asked about the “contagiousness” of a sick person, they answer this way: a sick person is not “contagious”, and the virus is just something that provokes the development of the disease in an organism that is genetically predisposed for this.

Varieties and symptoms

Pemphigus vulgaris (common)

It comes across more often, its share is up to 75% of all such cases of the disease. First of all, signs of the disease appear on the mucous membrane of the oral cavity and pharynx, subsequently spreading over the skin of the body: limbs, face, external genitalia. Observations show that dense blisters, small in size, can suddenly appear on apparently healthy skin, they quickly take on a sluggish appearance and are filled with a transparent serous fluid, which then becomes cloudy. Following the opening, erosive surfaces are formed, they then heal and traces remain in their place - brown pigment spots. Severe forms of the chronic form of the disease are also known. In practice, there are cases when, even in the absence of treatment, the condition spontaneously improves, and then an exacerbation occurs.

Be sure to remember about the likelihood of a secondary infection (for example, candidiasis). Due to the large loss of fluid, protein, secondary infections and their consequences, it is very difficult to give a positive prognosis in a severe form of the disease.

There are several types of bladderwort. Among the rare forms that differ in clinical manifestations, there are medicinal, paraneoplastic, herpetiform and other types of pemphigus. Different forms of this disease differ in the localization of the first blisters, the course of the disease, and the prognosis of treatment.

Vegetative pemphigus

It is a very rare form of this group of diseases. It can be confused with pemphigus vulgaris because they are similar in their first symptoms: blisters appear on the mucous membrane in the oral cavity. In the further course of the disease, there are already differences. With this rare form, blisters spread around natural openings (for example, the navel), in large skin folds (armpits, groin, between the buttocks, under the breast, behind the ears).

Areas of the skin covered with erosions due to the opening of the blisters are covered with papillomas. If we talk about the prognosis, then a favorable one is extremely rare.

Pemphigus foliaceus

This type of pemphigus is mainly characteristic of childhood. With this form, the blisters appear on the scalp, often on the back and chest. The first bubbles always look fragile and slightly raised, they are short-lived and burst quickly. Here we must talk about the reappearance of blisters, and under the crusts on the skin that has not yet healed.

seborrheic pemphigus

It is very rare, the specificity of the disease in its long-term course, but mostly it is benign. First, blisters appear on the skin of the face, chest, back, scalp. When crusts form, erosive places appear under them. Very often, the presence of pustules under the skin is noted. In the oral cavity are formed extremely rarely.

Methods of transmission of pemphigus vulgaris

Very experienced doctors testify that close relatives and children of a sick person can get sick. This is due to the fact that a defective gene can be transmitted to them, which causes changes in the epidermis, and also provokes the production of antibodies to this gene.

Stages of manifestation of the disease

initial stage

In most cases, pemphigus vulgaris appears in the oral cavity, pharynx, lips, lateral surfaces of the tongue are affected: first, blisters with a thin membrane appear, quickly bursting and provoking erosion (bright red or pale pink areas on the mucous membrane). Sometimes you may encounter halitosis: increased salivation and the appearance of cracks in the corners of the mouth. After some time, the symptoms of the disease appear on the skin. Most often on the chest, in the armpits, on the limbs, on the back, on the skin of the face, genitals. Bubbles are quite dense, sometimes with a red halo around. These blisters are filled with a transparent composition, subsequently suppuration is possible. When opening the bladder cavity, erosive phenomena occur on the skin. Erosive areas may be covered with a clear or purulent fluid, which forms crusts when dried. Subsequently, the healed place becomes a dark spot. The duration of the initial stage is 2-3 weeks, sometimes lasting several months. This stage of the disease is characterized by the absence of complaints about an uncomfortable condition: a person is usually only interested in appearance.

Generalization

At this time, the number of blisters increases everywhere (on the skin, in the oral cavity), their size is 1-40 mm. These places are subsequently covered by extensive erosion. If erosions are located on the red border of the lips, as well as in the mouth, they are very painful, can merge into a single space, create severe pain while eating and speaking. General health worsens: insomnia, lethargy, fever, pain when moving. Secondary infection appears just at this stage:

fungus: itching is felt on the erosive areas, they are covered with a white, “curd” coating, if it is removed, swelling and redness are visible, a high temperature rises;
bacterial infection: the fluid inside the bladder is yellowish, cloudy, the presence of edema, a high temperature is characteristic.

The sensation of the patient will depend on the location of the blisters. Finding them in the larynx will cause a rougher voice. If the location is the nose, then breathing is difficult, and there are always crusts in the nose (if they are removed, bleeding is possible).

At this stage, treatment with corticosteroids is indicated, if this is not done, then intoxication and exhaustion (cachexia) can lead to death. When using these drugs, the disease will move to the next stage.

epithelialization

This stage is characterized by the healing of erosions, the rarer appearance of new blisters, and they are becoming smaller in size. General well-being improves, only an imperceptible burning sensation remains and the skin slightly “tingles”.

Symptoms of mucosal lesions

First, blisters of light serous filling appear on the mucosa. Often a sick person ignores such blisters, because they burst imperceptibly for him, forming round or oval erosions. These affected areas are glossy, red in color, painful on palpation. Along the edges of the erosive area, the light edges of the upper layer of the epithelium are visible, this is what remains of the burst bubble. When pulling the edge of the epithelium over the edge with tweezers, it will also exfoliate on the mucous membrane, which does not have external painful symptoms (Nikolsky's symptom).

The patient feels a pronounced pain, notes an increased secretion of saliva. During this period, eating and caring for the oral cavity is quite painful. Subsequently, the addition of a secondary infection can be noted, purulent plaques, the presence of large bloody crusts on the lips, and the appearance of an unpleasant odor may appear. The voice may become hoarse when the lining of the larynx is affected.

Skin lesions: symptoms

On the outwardly clean and healthy skin of the body, the heads of the limbs, blisters appear, filled with a light liquid, gradually becoming cloudy. There is no regularity in the appearance of bubbles, they appear quite chaotically. The surface of the bubble is not tight, if the bubble is large, then it is flattened under its own weight in the lower part (this is called a pear symptom). We can talk about the addition of purulent microflora, since the contents are yellow and the presence of inflammation around the bladder. If such a bubble is opened, then red erosion with a bottom covered with epithelium can be observed. The edges of erosion are characterized by a rim consisting of the remnants of this bubble. Areas that are subjected to either pressure or friction have erosion that is constantly increasing in size. Such a surface is gradually covered with crusts (serous, purulent). At some point, the body temperature rises sharply, appetite disappears, sleep is disturbed, and pain appears. This is an indicator of intoxication of the body (occurs with a large area of \u200b\u200bdamage to the skin).

Special diagnostics: procedure

The diagnosis of this disease can only be made by a histological examination of the skin, which is performed using special forceps. This manipulation is performed under local anesthesia. A piece of skin is examined in the place where the bladder is fresh and small. An immunofluorescence reaction with a biopsy specimen is often carried out; during this manipulation, accumulations of immunoglobulin G can be detected on the membrane of epidermal cells and in the intercellular space. In the blood, this method can detect the presence of antibodies to desmosomes.

When the disease is concentrated in the mouth, a cytological examination is appropriate to determine the presence or absence of Tzank cells. If the result of this examination is positive, it is still necessary to conduct an additional histological examination.

Additionally, for the correct diagnosis, immunological methods are used that either confirm or deny the autoimmune nature of the disease. Direct immunofluorescence reaction (RIF) reveals accumulations of IgG in the intercellular space, as well as on the surface of the membranes of epidermal cells. Indirect RIF is carried out using the patient's serum and the presence of antibodies to the desmosomes of epidermal cells is determined.

What should be distinguished from vulgar pemphigus

Diagnosis allows you to distinguish this disease from the following ailments:

herpes;
allergy;
pemphigoid;
herpes zoster;
pemphigus of the eye;
flu-like stomatitis;
aphthous stomatitis;
vegetative pemphigus;
herpetiform dermatitis;
seborrheic pemphigus;
pemphigus Senir-Usher;
multiform exudative erythema;
family pemphigus Gougereau-Haley.

With this disease, blisters, which are the main symptom of the disease, may appear on the skin only a few months after they appear in the oral cavity, and may appear at the very beginning of the disease. Such blisters appear on visually healthy skin, they are filled with a clear liquid, characterized by the absence of itching and pain. Sometimes you can see redness of the skin around the bladder (thin rim). This disease is characterized by rash of blisters in foci in different places: on the chest, back, under the arms, in the groin. Then new blisters appear, capturing previously undamaged areas of the skin. The diagnosis is made by a dermatologist only on the basis of symptoms and instrumental examination data.

Treatment of pemphigus vulgaris and prognosis for recovery

The correct and only method of treatment with a comforting prognosis is the appointment of a high dose of corticosteroids (dexamethasone, triamcinolone, prednisone.) The initial dose of the drug determines the severity of the patient's condition. It is possible to reduce the dosage only after the epithelialization of all erosions begins and new rashes stop. If the treatment is successful, then the selection of a maintenance dose begins (the minimum amount of the drug per day, which prevents the appearance of fresh blisters). This dose is subsequently taken by the patient constantly and systematically.

Along with taking corticosteroids, treatment with cytostatics is prescribed: methotrexate, cyclosporine, azathioprine. This ensures the treatment of pemphigus with lower doses of corticosteroids, remission in the disease is achieved much faster. The use of methods of extracorporeal hemocorrection (plasmophoresis, hemosorption, etc.) ensures the purification of the blood from immune complexes and antibodies, with which the blood is saturated. All this allows us to talk about the remission of pemphigus vulgaris (very important in the case of low effectiveness of corticosteroids). Treatment of pemphigus vulgaris is carried out with potassium preparations, anabolic steroids, and in the case of a secondary infection, antibiotics. In order to avoid complications in the treatment of corticosteroids, you need to use drugs that protect the walls of the stomach (bismuth nitrate, etc.).

A positive outcome depends on the timeliness of treatment with corticosteroids. But the constant intake of these drugs for many years will necessarily lead to serious complications (internal organs and systems), which can cause the death of the patient.

Medicines

You can not do without a glucocorticoid (prednisolone). This drug has no contraindications that, given the specifics of the disease, would force doctors to refuse it. Treatment begins with shock doses of prednisolone: up to 90-120 mg per day. When the treatment takes place within a week, but the appearance of blisters cannot be avoided and erosion does not occur, the dose is increased by 1/3. In severe cases of the course of the disease, the initial dose can be doubled. When a positive trend occurs, loading doses are canceled and the amount of prescribed medication is gradually reduced. Drugs can be prescribed in tablet form: triamcinolone, methylprednisolone, prednisolone, kenacort, etc. In this case, you can reach the minimum dose at which new bubbles do not occur. This dosage is considered maintenance, prescribed for life.

Reception of cytostatics is possible only with the appointment of a doctor, it is impossible to self-medicate! They are used only in combination with glucocorticoids. When taking cytostatics, it is possible to reduce the dose of glucocorticoids and improve the prognosis of treatment. In this case, treatment is carried out with azathioprine, methotrexate, cyclosporine, cyclophosphamide.

Possibility of local treatment

For local (local) treatment use:

Solcoseryl and Actovegin, which improve metabolism, thanks to their intake, tissues heal faster.
Aerosol containing drugs such as oxytetracycline and hydrocortisone.
Ointment with hydrocortisone.
Medicine Triderm (a modern drug that combines a glucocorticoid, an antifungal agent in combination with an antibiotic).
Erythromycin ointment.

Prognosis: pemphigus vulgaris

In the absence of proper and systematic treatment, a sick person can die in 1-2 years, since purulent complications that are constantly expanding, sepsis and cachexia kill the body.

Therefore, self-treatment, as well as self-treatment with the help of traditional medicine, is strictly prohibited!

It is disappointing, but even with timely treatment, no one will give a prognosis for a complete recovery. This disease is not completely cured even with the help of treatment with corticosteroids and cytostatics. However, relapses (their frequency), their severity can definitely be reduced.

With an exacerbation, a long-term disability may occur, often patients receive a disability.

But enough episodes are known when, on the contrary, good control is achieved and a person can live a long time while maintaining a high quality of life.

With pemphigus vulgaris, there is often an increase and merging of erosions with the formation of significant lesions in the area. Diagnosis is carried out by biopsy of the newly appeared bladder, its histological and immunological study. In the treatment of pemphigus vulgaris, corticosteroids, cytostatics, methods of extracorporeal hemocorrection are used.

Pemphigus vulgaris

Pemphigus vulgaris is the most common clinical form of pemphigus. Its incidence in the world is 0.1-0.5 of the population. Usually older people get sick. Like other forms of pemphigus, pemphigus vulgaris refers to bullous dermatoses, since its main element is a bladder. About 2/3 of cases of the disease begin with the appearance of blisters on the oral mucosa, and only after a few months the skin is involved in the process.

Causes of pemphigus vulgaris

The development of pemphigus vulgaris is associated with disturbances in the functioning of the immune system, which results in the production of IgG antibodies to the own cells of the spiny layer of the epidermis. Under the action of autoantibodies, the destruction of desmosomes that bind epidermal cells to each other occurs. The loss of connections between cells (acantholysis) leads to the fact that the space between them is filled with intercellular fluid with the formation of acantholytic blisters characteristic of pemphigus vulgaris.

Symptoms of pemphigus vulgaris

Most often, pemphigus vulgaris begins with the mucous membrane of the mouth and throat. Due to mechanical damage from food, the blisters burst so quickly that they are almost never seen. In the mouth, against the background of unchanged mucosa, bright red painful erosions are formed. Fragments of a burst bubble covering erosion create a picture of a whitish coating on it, but they are easily removed with a spatula. Gradually, the number of erosion increases. Without specific therapy, they do not heal, but rather grow and merge. Due to severe pain, the patient cannot eat or talk. There is a fetid odor from the mouth.

A few days after their appearance, the bubbles open. The resulting erosions have a bright pink color. Gradually increasing in diameter, they merge and occupy vast areas of the skin. The general condition of the patient, not disturbed at first, with pemphigus vulgaris becomes worse, low-grade fever and weakness occur. Severe pain prevents active movements. When an infection is attached, pyoderma develops: the fluid in the blisters becomes cloudy, the erosions become covered with purulent discharge, and the patient's condition deteriorates sharply. Increasing cachexia or sepsis (with the addition of an infection) can lead to the death of the patient.

In the clinical diagnosis of vulgar pemphigus, mechanical symptoms are important, indicating acantholysis. These include Nikolsky's symptom - exfoliation of the epidermis with light friction of a healthy-looking area of \u200b\u200bthe skin. The marginal symptom of Nikolsky is checked by sipping on a piece of the wall of a burst bladder. If it is positive, then there is a peeling of the epidermis at a fairly large distance from erosion. Symptom Asbo-Hansen - pressing a finger on the bladder with pemphigus vulgaris leads to exfoliation of the epidermis along the periphery of the bladder and an increase in its area.

To confirm the diagnosis of pemphigus vulgaris, a cytological study is performed according to the Tzank method. Microscopy of a smear-imprint obtained from the bottom of the erosion reveals characteristic acantholytic cells in the spinous layer of the epidermis. For histological examination, during a biopsy, a skin area containing a fresh bladder is taken. The study reveals a cavity located inside the epidermis and determines the mechanism of its occurrence.

Additional in the diagnosis of pemphigus vulgaris are immunological methods that confirm or refute the autoimmune nature of the disease. Direct immunofluorescence reaction (RIF) reveals accumulations of IgG in the intercellular space and on the membranes of epidermal cells. Indirect RIF is carried out with the patient's serum and determines the presence of antibodies to the desmosomes of epidermal cells in it.

Treatment and prognosis of pemphigus vulgaris

The only way to save a patient with pemphigus vulgaris is to prescribe high doses of corticosteroids: dexamethasone, triamcinolone and prednisolone. The initial dose of the drug depends on the severity of the patient's condition. Reducing the dosage begins only after the epithelialization of all erosions and the cessation of fresh rashes. Gradually, a maintenance dose is selected - the minimum daily amount of the drug, when taken, the appearance of new rashes is not observed. The patient must take this dose continuously.

Together with corticosteroid therapy, cytostatics are used: methotrexate, cyclosporine, azathioprine. This allows for the treatment of pemphigus vulgaris with lower doses of corticosteroids and faster remission of the disease. The use of methods of extracorporeal hemocorrection (plasmophoresis, hemosorption, etc.) makes it possible to purify the blood from immune complexes and antibodies circulating in it. This contributes to the remission of pemphigus vulgaris and is especially important when the effectiveness of corticosteroid therapy is poor. In the treatment of pemphigus vulgaris, potassium preparations, anabolic steroids are used, and antibiotics are used when an infection is attached. To prevent complications of corticosteroid therapy, medications are prescribed that protect the stomach wall (bismuth nitrate, etc.), and other drugs.

Timely initiated therapy of pemphigus vulgaris with corticosteroids, as a rule, avoids death. However, long-term use of these drugs inevitably leads to severe complications from the internal organs and systems, which in turn can cause the death of the patient.

What is pemphigus vulgaris and is it contagious?

Pemphigus vulgaris is a rather dangerous and rare autoimmune disease. This disease is considered potentially dangerous and affects men and women equally. Usually, pemphigus vulgaris is recorded in middle-aged patients; no cases of damage to the child's body have been recorded. To know what methods of treatment to use against such an ailment, it is necessary to understand its nature and know how it can be transmitted. In the medical community, the disease is called pemphigus. Pemphigus is divided into vulgar, vegetative, leaf-shaped and seborrheic.

1 Nature of disease

Pemphigus vulgaris can occur against the background of the patient's complete health. A potentially healthy person notices the appearance of characteristic blistering formations on the skin and mucous membranes. The bubbles are filled from the inside with a clear liquid, which begins to become cloudy over time. Over time, these formations also begin to expand, spreading over the surface of the body. Upon reaching a certain size or due to mechanical damage, the bubble bursts, leaving the inflamed areas of moist skin in place. Thus, an ideal environment is formed for the reproduction of bacteria and infection, since the affected areas of the skin do not have a sufficient protective function.

Treatment must be carried out at the stage when the spread of blisters has not yet begun. To do this, use drugs that directly affect the immune system. If you start the disease, it can lead to extremely unpleasant consequences for health. Since open blisters tend to lead to suppuration, pemphigus vulgaris usually has a poor prognosis.

Pemphigus vulgaris, according to statistics, is observed in 1 case in 5 million patients. It occurs most often in the Mediterranean and Indian peninsula. This is due to the fact that these peoples often have consanguineous marriages that lead to genetic pathologies, including the structure of the gene that is responsible for the condition of the skin.

Thus, it can be concluded how the disease is transmitted. It is impossible to get infected from a sick person with this disease. But the genes that are passed on to children can provoke the onset of pathology.

2 Disease in terms of physiology

The human epidermis has a special structure, which determines the nature of the disease.

The epidermis consists of four main layers throughout the body, except for the palms and soles, where there are 5 of these layers. The two lower layers are connected by special processes that have a protein character. They are called desmosomes. It is these processes that are responsible for the strength of the skin. Between themselves, they create special connections like carbines.

The next 2 layers do not have such connections and protect the deep layers from mechanical, thermal and chemical damage.

With the manifestations of pemphigus, the human immune system begins to produce antibodies to protein compounds that destroy desmosome compounds. It is the detection of these antibodies, which are called Tzank cells, by biopsy that makes it possible to make a diagnosis.

In the process of detaching the skin layers, a special liquid is formed, it is she who fills the bubbles that appear on the surface of the skin or mucous membrane. The area of appearance of bubbles is limited to damaged desmosomes, where the cells are not damaged and the border of bubbles appears. The top layer of the bladder is the protective layers of the skin. If the bubble bursts, then the lower skin layers remain defenseless against external influences. In addition, the fluid contained in the bladder was saturated not only with protein compounds, but also with electrolytes and microelements. Consequently, the body also loses these substances in the process of spreading the disease through the skin.

3 Causes of pathology

Scientists have not fully figured out the cause of this phenomenon. In addition to changing the structure of the gene that is responsible for the proper structure of the epidermis, experts identify some possible causes that can trigger the development of the disease:

violations of the water and electrolyte balance in the body (a large amount of fluid is retained in the patient's body or salts accumulate);
some serious diseases of the nervous system can disrupt immune function;
diseases of the adrenal glands that disrupt their functionality in the human endocrine system;
violation of basic biochemical reactions due to improper functioning of enzymes;
exposure to sunlight, chemical reactions and exposure to aggressive components of some drugs.

However, even these factors are not capable of causing an illness on their own, only an organism genetically predisposed to it can react with a manifestation of the disease.

Scientists also put forward the theory of the viral origin of pemphigus. This theory is based on the assertion that the disease is of a viral nature, but the virus itself remains undetected. Even within the framework of this theory, pemphigus remains not contagious, since the virus can only provoke changes in a predisposed organism.

4 Symptoms and characteristic manifestations

Since the disease can develop even against the background of the complete health of the patient, there are several stages in the development of the pathology.

This stage is characterized by the appearance of small bubbles with a thin top layer. They are localized in the face, often around the lips. The top layer of bubbles is rapidly destroyed, leaving erosion in its place. Further, pemphigus begins to spread over the skin, new foci appear, where the blisters are localized. Most often they begin to appear in places with a thin layer of the epidermis:

armpits;
genitals;
breast;
inner thighs;
back.

In addition to cosmetic, this stage does not cause any discomfort for the patient. After the destruction of the upper layer of blisters, areas of the skin affected by erosion heal quickly. The skin after that has a slightly darker shade.

At this stage, not only the number of bubbles increases, but also their size. Almost the entire surface of the body is affected here, blisters often appear in the mouth, begin to merge with each other. There may be the following symptoms:

insomnia;
increased fatigue;
depression and depression;
heat;
fungal infections;
bacterial infections.

If you do not start treatment, then such a condition can even provoke the death of the patient.

The right treatments will take pemphigus to the next stage.

Epithelization.

Erosions gradually heal, leaving behind dark marks. The patient's condition returns to normal, the skin acquires a healthier appearance.

In any case, do not let the disease take its course. When the first symptoms appear in the form of localized blisters, you should immediately contact a specialist for further diagnosis and qualified treatment.

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Pemphigus vulgaris - causes, symptoms, treatment

Definition

Most often manifested in people of age, but cases of occurrence even in young children have been described. If you do not carry out adequate therapy for 1-2 years, it can lead to death.

Causes of true pemphigus

Cases of the development of the disease after pronounced exposure to UV rays, burns, and taking certain medications are described.

Often, pemphigus occurs along with other autoimmune processes. Therefore, we can conclude that the disease is not contagious and cannot be transmitted to other people.

Symptoms

Somewhat less commonly, the mucosa of the posterior wall of the pharynx, larynx, rectum, genital organs, and nose is primarily affected. Gradually, the rash spreads to the rest of the skin.

Localization of blisters with a typical onset of pemphigus vulgaris:

area near the pharynx
lateral surfaces of the tongue
soft and hard palate
mucous membrane under the tongue
gum mucosa
inner surface of both lips

Symptoms of mucosal lesions

Initially, blisters filled with light serous contents are found on the externally unchanged oral mucosa.
More often, a person does not notice blisters, because they quickly open up and form oval or round erosions. The lesions have a glossy red surface, are quite painful on palpation
On the periphery of erosion, light fragments of the surface layer of the epithelium are visible - the remains of an opened bladder. If you gently pull the tweezers over the edge, then the epithelium will exfoliate on the outwardly unchanged mucosa (Nikolsky's symptom)
The patient is concerned about severe pain, increased salivation. Very difficult to eat and take care of your mouth
A secondary infection gradually joins, purulent plaques appear on erosions, large bloody crusts on the lips, and an unpleasant odor.
Hoarseness of voice - occurs with damage to the mucous membrane of the larynx
If the nasal cavity is affected, then constantly drying crusts make breathing difficult and cause frequent bleeding.

Symptoms of skin lesions

It all starts with the appearance of blisters on the unchanged skin of the trunk, head, extremities of blisters with light content, which gradually becomes cloudy. They are randomly distributed on the body. The cover of the blisters is quite flabby, and the largest of them stretch in the lower part under their own pressure - a symptom of a pear
The yellow color of the contents and inflammation around the element indicate the addition of pyogenic microflora
After opening the bubble, red erosion is formed, the bottom of which is slowly covered with epithelium. Along the edges of erosion - a border of the remains of the bubble
In areas where the skin is subject to friction and pressure, the size of erosions gradually increases. They are slowly covered with serous and purulent crusts.
When the skin lesion reaches a large scale, the body undergoes intoxication, which is manifested by fever, lack of appetite, sleep disturbance and severe pain.

If adequate therapy is not carried out, cachexia or sepsis may occur, which will lead to the death of the patient.

Diagnosis of vulgar pemphigus

It all starts with an examination by a dermatologist, who assesses the person's condition, characteristic changes and prescribes additional examinations.

Symptom of the Nikolsky marginal - pulling a piece of the bladder cover with tweezers leads to exfoliation of the epithelium on the outwardly unchanged skin next to the erosion.
Symptom Nikolsky focal - if you rub the unchanged skin between blisters or erosions, then the upper layers easily peel off.
Asboe-Hansen's symptom - when pressing on the bubble from above with a finger or a glass slide, its area increases due to the exfoliation of the epidermis.

Treatment

Since the etiology of the disease is unknown, it is symptomatic.

Glucocorticoids

It all starts with loading doses of prednisolone, which reach mg per day. If the patient receives treatment for a week, and new blisters continue to appear and epithelialization of erosion does not occur, then it increases by a third. In the most severe cases, doubling the initial dose is possible.
When a positive effect is achieved, loading doses are canceled, and a gradual decrease in the amount of the drug begins. Tableted forms of glucocorticoids are used (prednisolone, methylprednisolone, triamcinolone, kenacort, etc.).
A minimum dose is reached at which no new elements appear. It is called maintenance and is taken by the patient on an outpatient basis for life.

Cytostatics

Other drugs

Local treatment

Aniline dyes (brilliant green).
Preparations such as Solcoseryl and Actovegin - improve metabolism and accelerate tissue healing.
Aerosol with hydrocortisone and oxytetracycline.
Hydrocortisone ointment.
Erythromycin ointment.
Triderm is a modern drug that combines a glucocorticoid, an antifungal agent and an antibiotic.

Prognosis for pemphigus vulgaris

With timely treatment, the prognosis is also as if unfavorable. The disease does not go away completely against the background of constant intake of corticosteroids and cytostatic therapy, but the frequency of relapses and their severity are somewhat reduced. In moments of exacerbation, a long-term disability is possible, often patients become disabled. In some cases, on the contrary, good control is achieved and the patient lives a fairly full life.

Pemphigus vulgaris

The statistics of such illnesses

The essence of the disease

The epidermis (upper layer of human skin) is characterized by a special structure. The epidermis consists of four layers, the palms and soles of the feet even have five layers. As part of the two lower layers - from fifteen or more layers. The cells in these layers are connected by special processes called desmosomes (they have a protein structure). Desmosomes are woven into cell membranes in a very strong network. Intercellular contacts even form a kind of "carabiners" that provide a strong and inextricable connection. This circumstance makes the skin durable, not tearing when rubbed or stretched.

Causes

There is still no consensus on the causes of this disease. There are various versions and assumptions about the causes of the disease:

Endogenous - genetic, associated with immunity;
Exogenous factors - exposure to drugs: penicillin and its derivatives, interferons;
Physical factors (impact of harmful factors) - burns, ultraviolet radiation, radiation;
Viruses - herpes;
Diseases of the nervous system;
Violation of water-salt metabolism: a large amount of salts in the body and, as a result, fluid retention in the body;
Endocrine - this is mainly due to adrenal dysfunction;
Changes in the structure of enzymes that are responsible for the main biochemical reactions;
Food.

When summarizing all versions, you can group them into the following groups:

associated with the immune system;
Endocrine causes;
Causes of the nervous system;
Factors associated with improper metabolism;
exposure to infection;
Influence of toxic substances.

Varieties and symptoms

Pemphigus vulgaris (common)

Be sure to remember about the likelihood of a secondary infection (for example, candidiasis). Due to the large loss of fluid, protein, secondary infections and their consequences, it is very difficult to give a positive prognosis in a severe form of the disease.

Vegetative pemphigus

Areas of the skin covered with erosions due to the opening of the blisters are covered with papillomas. If we talk about the prognosis, then a favorable one is extremely rare.

Pemphigus foliaceus

seborrheic pemphigus

Methods of transmission of pemphigus vulgaris

Stages of manifestation of the disease

initial stage

Generalization

fungus: itching is felt on the erosive areas, they are covered with a white, “curd” coating, if it is removed, swelling and redness are visible, a high temperature rises;
bacterial infection: the fluid inside the bladder is yellowish, cloudy, the presence of edema, a high temperature is characteristic.

epithelialization

Symptoms of mucosal lesions

Skin lesions: symptoms

Special diagnostics: procedure

When the disease is concentrated in the mouth, a cytological examination is appropriate to determine the presence or absence of Tzank cells. If the result of this examination is positive, it is still necessary to conduct an additional histological examination.

What should be distinguished from vulgar pemphigus

Diagnosis allows you to distinguish this disease from the following ailments:

herpes;
allergy;
pemphigoid;
herpes zoster;
pemphigus of the eye;
flu-like stomatitis;
aphthous stomatitis;
vegetative pemphigus;
herpetiform dermatitis;
seborrheic pemphigus;
pemphigus Senir-Usher;
multiform exudative erythema;
family pemphigus Gougereau-Haley.

Treatment of pemphigus vulgaris and prognosis for recovery

Medicines

You can not do without a glucocorticoid (prednisolone). This drug has no contraindications that, given the specifics of the disease, would force doctors to refuse it. Treatment begins with shock doses of prednisolone: domg per day. When the treatment takes place within a week, but the appearance of blisters cannot be avoided and erosion does not occur, the dose is increased by 1/3. In severe cases of the course of the disease, the initial dose can be doubled. When a positive trend occurs, loading doses are canceled and the amount of prescribed medication is gradually reduced. Drugs can be prescribed in tablet form: triamcinolone, methylprednisolone, prednisolone, kenacort, etc. In this case, you can reach the minimum dose at which new bubbles do not occur. This dosage is considered maintenance, prescribed for life.

Possibility of local treatment

For local (local) treatment use:

Solcoseryl and Actovegin, which improve metabolism, thanks to their intake, tissues heal faster.
Aerosol containing drugs such as oxytetracycline and hydrocortisone.
Ointment with hydrocortisone.
Medicine Triderm (a modern drug that combines a glucocorticoid, an antifungal agent in combination with an antibiotic).
Erythromycin ointment.

Prognosis: pemphigus vulgaris

In the absence of proper and systematic treatment, a sick person can die in 1-2 years, since purulent complications that are constantly expanding, sepsis and cachexia kill the body.

Therefore, self-treatment, as well as self-treatment with the help of traditional medicine, is strictly prohibited!

With an exacerbation, a long-term disability may occur, often patients receive a disability.

But enough episodes are known when, on the contrary, good control is achieved and a person can live a long time while maintaining a high quality of life.

How to treat pemphigus vulgaris

Pemphigus vulgaris or pemphigus is a severe autoimmune disease in which skin lesions occur. The disease got its name for the reason that blisters form on the patient's skin. These bubbles are filled with fluid. Over time, they burst, and the skin becomes ulcerated. At present, the causes that provoke the onset of pathology have not been precisely established. It is believed that the disease can be triggered by a number of factors that affect human immunity. The disease is very dangerous and without proper treatment leads to death. The patient dies of exhaustion or concomitant pathologies. Often, bacteria begin to develop on the erosive surface, an infectious process occurs that can develop into sepsis.

For the treatment of the disease, folk remedies are used. This treatment is safe and does not cause side effects. Treatment with folk remedies is aimed at improving the condition of the skin, preventing the development of a bacterial infection and healing wounds. In addition to external agents, they also take herbal decoctions, which reduce the inflammatory process and improve the general condition of the patient.

Causes of the disease

Most often, pemphigus vulgaris develops in women over 30 years of age. Residents of Israel, India and Mediterranean countries are more susceptible to this disease. It is possible that solar radiation can provoke the onset of the pathological process.

Pemphigus vulgaris is an autoimmune disease. This means that during this pathological process, antibodies to their own tissues are formed in the human body. In the case of pemphigus, antibodies affect proteins in human skin cells that are responsible for intercellular communication.

The exact causes of pemphigus are currently unknown. There are a number of negative factors that, according to doctors, can provoke the development of the disease.

diseases of the nervous system, emotional disorders, stress, depression;
infectious diseases, in particular viral infections;
metabolic disorders, in particular, water-salt balance, fluid and salt retention in the body;
endocrine disorders, in particular, deterioration of the adrenal glands;
disruption of the immune system;
negative effect of external factors: ultraviolet radiation, thermal burns, damage to the integrity of the skin;
long-term use of certain drugs;
genetic predisposition.

Pemphigus is a non-communicable disease, and it is not transmitted from a sick person to a healthy one. Contact with sick people does not pose any danger. Sometimes the disease develops within the same family, but this is not due to the transmission of the disease from one person to another, but with the genetic predisposition of all family members to pemphigus.

Disease history

To understand the pattern of the development of the disease, it is necessary to know the structural features of human skin. The top layer of the skin is the epidermis. It consists of four main layers. Beneath them lie two layers of living cells. The cells of the first of these layers, the lowest, are interconnected by special cellular structures - desmosomes. This gives the skin strength and prevents tearing. Next is the granular layer. The cells of the granular layer are not interconnected by desmosomes. The top two layers are dead cells, their main function is protective. They protect the code from mechanical, thermal and chemical damage, microbial contamination.

When pemphigus in the human body are formed antibodies to the protein desmosomes. These structures are destroyed, which leads to cell separation. This process has a special name - acantholysis. Acantholysis proceeds with the formation of exudate, which is limited on the sides by intact skin cells, and on top - by keratinized epithelium. Thus, with pemphigus, a bubble is formed. The diameter of the neoplasm can be different: from a few millimeters to several centimeters.

The upper skin or lid of the bladder quickly bursts and ulceration forms. In fact, it is a wound surface that hurts. Often a secondary bacterial infection begins to develop in this place. Such an infectious process can become generalized, and sepsis develops. Also, this place is easily damaged by mechanical stress, thermal or chemical burns.

But the danger of the disease is not only in skin damage and the infectious process. The main danger lies in the depletion of the body. This depletion occurs because when the bubble bursts, a fluid rich in proteins and electrolytes flows out of it. This leads to the loss of substances necessary for the body. If the damage to the skin is extensive, it can be very difficult to make up for such a loss.

Symptoms of pathology

There are several stages in the development of pemphigus vulgaris.

Step one: start the process.

In 2/3 of sick people, the disease begins with damage to the oral cavity. Bubbles form on the mucous membrane of the mouth, pharynx, lower part of the tongue, and also on the skin around the mouth. The bubbles are covered with a thin skin and quickly burst. In their place, erosions occur, which stand out against the background of normal mucosa with their bright red color. The patient has increased salivation. The skin at the corners of the lips cracks.

After about a month, blisters begin to appear on the skin of the face, neck, arms, upper body and in the area of skin folds in the groin, under the breasts and armpits. Bubbles can form on the mucous membrane of the genital organs.

The skin over the blisters is stretched, they are filled with a liquid, initially transparent and gradually becoming cloudy. When the blisters burst, an ulceration forms, which then crusts over and dries up. The skin in this place becomes darker.

The duration of the first stage of the process ranges from several weeks to several months. The general state of health of the patient is satisfactory.

Stage two: generalization.

At this stage, the number of blisters increases both on the skin and in the oral cavity. The size of the blisters varies from 1 mm to 4 cm. When they burst, an erosive surface remains. Erosion can merge with each other. The person is in pain. Due to damage to the mucous membrane of the oral cavity, the process of eating and talking is difficult. Movement is also difficult due to skin lesions.

Often at this stage, an infectious process joins. Erosion can be affected by fungal and bacterial infection. At the same time, body temperature rises, erosive surfaces become covered with plaque, suppuration begins.

There is a general deterioration in the patient's condition: there is weakness, insomnia, fever. Symptoms of general intoxication of the body increase, exhaustion progresses. Without treatment, the disease is fatal. If therapy has been started, pemphigus moves on to the next stage.

Stage three: epithelialization.

Against the background of well-chosen therapy, there is a decrease in the manifestation of the disease. Bubbles occur less often, their size decreases. The pain syndrome is reduced, the general condition of the patient is normalized.

Diagnosis of the disease

Pemphigus vulgaris is diagnosed based on the clinical manifestations of the disease. To confirm the diagnosis, a histological examination of a sample of damaged skin is performed. He also conducts an immunological blood test, which detects the presence of antibodies to the skin's own proteins. A general laboratory blood test shows anemia and an inflammatory process.

Treatment of pemphigus vulgaris

Pemphigus vulgaris is a severe autoimmune disease that, if not properly treated, can lead to death of the patient.

Therapy of the disease is aimed at restoring the skin and speedy healing of wounds. It is also important to take anti-inflammatory drugs. There is a folk treatment for pemphigus. External folk remedies have analgesic, anti-inflammatory and antimicrobial effects. They prevent the development of a secondary bacterial or fungal infection and promote the speedy healing of wounds. Decoctions for oral administration have an anti-inflammatory effect and improve the overall health of a person.

External folk remedies:

People's drug. It is necessary to grind into gruel and mix equal amounts of garlic, onion, pepper, as well as salt and honey. Everything is thoroughly mixed and kept in the oven for 15 minutes. This mixture is cooled and stored in the refrigerator. The ointment is used to lubricate the wound surface twice a day.
Folk drug number 2. Crushed into gruel and mixed in equal proportions of lilac, wormwood, yarrow and plantain leaves. This gruel is applied to damaged skin and covered with gauze. The duration of the procedure is a quarter of an hour.
Nettle and aloe. The juice of these plants promotes wound healing, anesthetizes, relieves inflammation and prevents the development of infection. The use of aloe juice and nettle can be alternated. To prepare the drug, the leaves are crushed into gruel and the juice is squeezed out with the help of gauze rolled up in several layers. It is moistened with sterile gauze or cotton fabric, previously ironed, and applied to the wound surface. Fresh juice is prepared every time.
Vegetable oil. It is useful to lubricate damaged skin with olive, sunflower, corn, sea buckthorn or other vegetable oil. This helps separate dried crusts and also promotes healing.

With the development of the disease in the oral cavity, medicinal herbal decoctions are used for rinsing. Recipes:

Herbal collection. Mix in equal proportions of sage, chamomile and calendula. In half a liter of boiling water, steam 4 tbsp. l. such a collection, insist in a thermos for three hours, then filtered. The oral cavity is rinsed with warm decoction 3-4 times a day, every time after eating. Also, this remedy can be used to wash erosions on the skin.
Eucalyptus. In a glass of boiling water, steam 1 tsp. eucalyptus, insist in a thermos for 2 hours, then filter. Rinse the mouth with this infusion twice a day after meals.
Chamomile and mint. Dried grass is mixed in equal proportions and steamed in boiling water at the rate of 2 tbsp. l. for half a liter of water. Insist for two hours, then filter. Rinse your mouth 3-4 times a day after eating.

Drugs for oral administration:

Herbal collection No. 1. A mixture is prepared from two parts of eucalyptus, birch buds and chamomile, 3 parts of yarrow herb and 4 parts of St. John's wort. In half a liter of boiling water, steam 2 tbsp. l. such a collection, insist two hours, then filter. Use 50 ml of the drug every 4 hours. Therapy lasts at least three months.
Tansy. In a glass of boiling water, steam 1 tsp. tansy color, insist an hour, then filter. Drink 20 ml of infusion three times a day. Therapy lasts a week, then take a week break.
Clover. In 200 ml of boiling water, steam 2 tbsp. l. crushed clover heads, insist two hours and filter. Drink 50 ml 3-4 times a day.

The approach to treatment should be complex, and in therapy it is best to combine different means. Every three weeks it is necessary to change the drug so that addiction does not develop and the healing effect does not disappear.

Treatment of the disease includes maintaining a healthy lifestyle and proper nutrition. A person with pemphigus often loses his appetite, but he still needs to eat well to make up for the loss of protein and electrolyte with exudate. It is important to eat plenty of vegetables and fruits as sources of vitamins and minerals. Juices and herbal teas are suitable for drinking.

In case of damage to the oral cavity, food should be boiled or steamed. It is useful in this case to eat mashed soups and mashed cereals.

It is important to avoid hard physical labor, stress and maintain a positive attitude, fully relax and get enough sleep.

Write in the comments about your experience in the treatment of diseases, help other readers of the site!

2 Disease in terms of physiology

The human epidermis has a special structure, which determines the nature of the disease.

The next 2 layers do not have such connections and protect the deep layers from mechanical, thermal and chemical damage.

3 Causes of pathology

violations of the water and electrolyte balance in the body (a large amount of fluid is retained in the patient's body or salts accumulate);
some serious diseases of the nervous system can disrupt immune function;
diseases of the adrenal glands that disrupt their functionality in the human endocrine system;
violation of basic biochemical reactions due to improper functioning of enzymes;
exposure to sunlight, chemical reactions and exposure to aggressive components of some drugs.

However, even these factors are not capable of causing an illness on their own, only an organism genetically predisposed to it can react with a manifestation of the disease.

4 Symptoms and characteristic manifestations

Since the disease can develop even against the background of the complete health of the patient, there are several stages in the development of the pathology.

Initial stage.

armpits;
genitals;
breast;
inner thighs;
back.

Generalization.

insomnia;
increased fatigue;
depression and depression;
heat;
fungal infections;
bacterial infections.

If you do not start treatment, then such a condition can even provoke the death of the patient.

The right treatments will take pemphigus to the next stage.

Epithelization.

Erosions gradually heal, leaving behind dark marks. The patient's condition returns to normal, the skin acquires a healthier appearance.

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, the dermis. If these are opened, pink wounds will appear in their place.

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

Features of the disease

Pemphigus vulgaris is known in the medical community as the most common form of pemphigus. More often the disease occurs in older patients, whose age is within 30 - 60 years.

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

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

Pemphigus vulgaris (photo)

Causes

Usually, this disease occurs when the immune system is impaired. In this case, the body produces antibodies (IgG) to its own cells, which are localized in the prickly layer of the epidermis.

The desmosomes that bind epidermal cells are destroyed due to exposure to 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 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 hurt and have a characteristic bright red color remain. If you do not start treatment, there is growth, fusion of bubbles. At this stage of the development of the disease, the following symptoms are observed:

fetid odor from the mouth;
loss of appetite due to pain;
erosion on the oral mucosa.

On the epidermis, bubbles will begin to appear a few months after their formation on the oral mucosa. Very rarely it can be noted around the bubble. It is like a thin rim. Rashes in 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 a few days after their occurrence. The resulting erosions are distinguished by a bright pink color, large size, and a tendency to merge. The patient begins to worry about such signs:

the appearance of purulent discharge on erosions;
pain;
turbidity of the fluid accumulated inside the blisters;
(it can develop after infection).

Diagnostics

Mechanical symptoms indicating acantholysis are considered especially significant. Specialists can carry out the following procedures:

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

To confirm the alleged diagnosis, a cytological examination (Tzank's method) can be performed. Thanks to the microscopy of a smear taken from the bottom of the wound, it can be detected. These cells are present in the spinous layer of the epidermis. Material is taken from a fresh bladder.

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

Treatment

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

Therapeutic

Along with the use of medications, extracorporeal hemocorrection is prescribed. The most commonly used for blood purification are:

plasmaphoresis;
hemosorption.

Medical

Drug therapy involves the use of such groups of drugs:

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

Antibiotics are also needed in case of infection. In order to prevent complications that may occur 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").

Do not treat pemphigus with folk remedies without the permission of a doctor!

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 that are necessary to prevent relapse. They consist in:

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

Pemphigus vulgaris in the oral cavity

Complications

Due to the large number of side effects of glucocorticoids, serious complications are possible. Long-term use of these drugs can 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 the mental state (the occurrence of euphoria, manic-depressive);
exacerbation (peptic);
thrombophlebitis;
disruptions in the menstrual cycle;
failures in protein metabolism;
slowdown of recovery processes;
violation of fat metabolism;
spontaneous fractures (they occur due to decalcification of the bones);
decrease in immunity.

Forecast

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

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