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Is it possible to get dentures if you have pemphigus? Pemphigus vulgaris of the oral cavity

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 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;

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.

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.

Cause of pemphigus

Until now, doctors have not been able to determine what causes the blisters. There are autoimmune, toxic, bacterial, viral, neurogenic theories.

It has been proven that the cause of the destruction of intercellular connections is the aggression of the body’s own (autoimmune process), but what provokes it is unknown.

Possible root causes of the formation of pemphigus are disturbances in the functioning of the child’s immune system. 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 environmental conditions. Bubbles are formed due to disturbances in metabolic processes between cells.

The main factors provoking the disease are:

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

Reproduction of microorganisms under the dermis

Types of pemphigus

Depending on whether acantholysis (destruction of intercellular connections) occurs in the skin, all pemphigus is divided into true (acantholytic) and non-acantholytic (pemphigoid). In both cases, blisters appear on the patient’s body, which are very similar to each other.

Currently, doctors distinguish the following forms:

pemphigus vulgaris;
pemphigus vegetans;
Brazilian pemphigus is endemic and is found only in some peoples of Brazil;
pemphigus foliaceus;
erythematous pemphigus;
paraneoplastic pemphigus - provoked by cancer;
chronic congenital familial pemphigus (also known as Hailey-Hailey disease);
Dühring's dermatosis herpetiformis;
cicatricial pemphigoid;
bullous pemphigoid.

The group of blistering dermatoses includes the following diseases:

pemphigus classic or pemphigus;

pemphigus ocular or cicatricial pemphigoid;

bullous pemphigoid;

Dühring's dermatitis herpetiformis.

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.

With this disease, transformation from one form to another is often observed.

There are several types of pemphigus:

Vulgar, which occurs most often. Its main symptoms are blisters on the mucous membrane of the gums, cheeks, and palate. They quickly burst, and in their place painful red erosions form, which are bordered by the remains of a blister. Sometimes these wounds become covered with a whitish coating. Over time, blisters appear on the skin of the chest and back of a sick person. Moreover, they can be of different sizes. The blisters contain clear serous fluid. After a few days they dry out and become crusty. In some cases, the blisters burst and red erosions appear in their place. Medical history is important in treating this disease. Pemphigus vulgaris often appears in those whose parents suffered from this disease. Once a hereditary connection is established, it will be easier for the doctor to prescribe the most effective type of therapy.

Erymatous, in which blisters first appear on the skin. They form on the face, chest, neck, and scalp. At the beginning of the disease they are seborrheic in nature. The bubbles have clear boundaries, and their surface is covered with yellow-brown crusts. When they are separated, the eroded surface of the skin is revealed. Erymatous pemphigus is a disease that experts differentiate from seborrheic dermatitis or lupus erythematosus.

Leaf-shaped, which manifests itself as erythema-squamatous rashes. With it, thin-walled blisters appear on previously affected areas of the skin. Once opened, a red, eroded surface is exposed. When it dries, lamellar crusts form. With this form, blisters may reappear directly on them. Because of this, a thick layered crust forms on the skin. There is a constant separation of exudate.

Vegetative, which is characterized by a sluggish course. With it, blisters most often affect the skin around openings on the body and in the area of skin folds. After opening them, erosions with a foul odor remain. Vegetations (pathological growths of tissue) appear on them, which are covered with serous-purulent plaque.

Symptoms

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, there is increased salivation and, characteristically, bad breath. Patients often turn to a dentist or otolaryngologist with such symptoms and are unsuccessfully treated for stomatitis, rhinitis or laryngitis.

As can be assumed from what was written earlier, so many forms of the disease have been identified due to the wide variety of manifestations and course of the disease. Basic information on each variety will be briefly presented below.

Pemphigus vulgaris (acantholytic)

In patients, against the background of absolutely normal epithelium, large blisters form in the oral cavity and in the pharynx area. They quickly open spontaneously or as a result of damage, revealing a large area of erosion, along the edges of which fragments of the epithelium remain.

Erosions on the lips are covered with large hemorrhagic crusts. For several months, patients may experience exclusively lesions in the oral mucosa. They turn to dentists, who often mistake pemphigus for stomatitis.

Afterwards, blisters appear on the previously unchanged skin of the back and chest. Particularly large elements under their own weight can take on a pear-shaped shape. The contents of the bladder are transparent serous.

Pemphigus vegetans (in the oral cavity)

With this form, blisters can appear not only on the oral mucosa and open areas of the body, but also in the armpits, in the groin, near natural openings, and in the folds under the mammary glands.

After their opening, erosions form, in place of which growths of the papillomatous type gradually form (hence the name). They gradually resolve on their own, leaving behind an area of hyperpigmentation.

If proper measures are not taken, infectious complications quickly develop, which worsens the patient's condition.

Pemphigus foliaceus

It got its name because of the appearance of the bubbles. They are very flat, but occupy a large area. There is relatively little content inside, which is why the elements are quite sluggish.

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

weakness;
fatigue;
drowsiness;
loss of appetite;
may be: runny nose, sore throat, headache, cough, and sometimes loose stool.

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 avoiding spicy and spicy foods 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.

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.

Despite the common symptom in the form of a blistering rash, different diseases have their own, characteristic only for them, manifestations.

Pemphigus

This is a classic version of pemphigus. The essence of the disease is the appearance of blisters from exfoliated epidermis on the inflamed skin. Mostly people over 35 years of age get sick; children very rarely get sick.

The first rashes appear on the mucous membrane of the oral cavity, in the pharynx. Gradually the rash spreads to the entire body.

Pemphigus vesicles on the mucous membrane are very thin and burst easily. In their place, erosive changes form.

As a result, eating and even talking are very difficult due to pain.

Pemphigus vesicles on the skin are more durable, but they also open with the formation of erosions. They occupy vast areas. Then the erosions are covered with dense crusts, after the removal of which foci of pigmentation remain.

Note. The disease can have a benign course, in which the patient’s condition practically does not worsen. There is also a malignant course with severe intoxication, exhaustion and dehydration.

According to the characteristics of symptoms, four forms are distinguished:

pemphigus vulgaris;

pemphigus foliaceus;

seborrheic pemphigus, or erythematous;

vegetative.

With pemphigus vegetans, the rash tends to be located in skin folds, around the natural openings of the body and the navel. After the blisters open, instead of erosions, skin growths appear - vegetations.

They have a grayish color. Papillomas can merge and form extensive lesions.

The growths produce abundant fluid. Patients experience severe pain and itching.

The leaf-shaped form of pemphigus is often found in children. Bubbles with this option consist of several layers of the epidermis. After opening them, scaly crusts form on the skin.

Important. This disease lasts for many years, gradually spreading to the entire skin, including the scalp. The larger the lesions, the worse the patient's condition.

A type of pemphigus foliaceus is Brazilian or epidemic pemphigus. It often affects all members of the same family. The disease is widespread in South America. There is a high probability that this pemphigus is infectious, but its causative agent has not yet been identified.

Seborrheic pemphigus is called Senir-Usher syndrome. By its origin, this is true pemphigus - it can develop into other variants of pemphigus.

The main part of the rashes is localized on the skin. If bubbles appear on the mucous membrane, this is an unfavorable sign.

The blisters are practically invisible; they immediately become covered with yellow crusts, as with seborrhea.

Pemphigus eye

The disease is typical for women over 50 years of age. First, conjunctivitis develops - unilateral or bilateral.

Then, against the background of the inflamed conjunctiva, thin blisters form. After opening them, adhesions are formed, which lead to the fusion of the eyelids with each other.

The eyeball becomes motionless and blindness develops.

Note. In addition to the conjunctiva, pemphigus vesicles appear on the oral mucosa. There they are dense and tense. After opening them, deep painful erosions form.

Bullous pemphigoid

Bubbles appear on symmetrical areas of the body - on the sides of the body, the inner surfaces of the thighs. The background may be unchanged or hyperemic skin. Some blisters contain hemorrhagic contents. The rash is accompanied by itching.

The blisters can coalesce and reach several centimeters in diameter, forming bullae.

Dühring's dermatitis

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.

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 heaviness of the contents. Soreness of pemphigus foci is not always noted, as is itching.

However, the resulting erosions always cause a lot of unpleasant sensations. Due to the 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 is 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 pain. After 2-3 weeks, a new nail plate grows, so there are no external traces of the transferred pemphigus.

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.

Diagnostics

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.

Using an immunological study, deposits of IgG and IgA can be detected between the epidermal cells in the area of the bladder.

The diagnosis is made based on:

Diagnosis of pemphigus The diagnosis of true pemphigus is established based on the following methods.

The diagnosis is based on a characteristic clinical picture. Confirmation is a histological examination of the epidermis. If acantholysis is detected, the diagnosis of pemphigus is considered reliable.

Pemphigoids - bullous and cicatricial - are diagnosed based on the characteristic clinical picture. Histological examination of the epidermis does not reveal acantholysis of the stratum spinosum.

Dühring's dermatitis is diagnosed based on the typical clinical picture. Of the specific methods, the Jadasson test is used - when an iodine solution is applied to the skin, the number of rashes increases.

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

At the initial stage of this disease, only an experienced specialist can determine that it is pemphigus. The medical history in this case can cover a very long period of time, since its course can be quite long.

At the initial stages, the clinical manifestations of pemphigus are uninformative, so the doctor carefully interviews the patient.

To confirm the diagnosis, laboratory tests are performed:

Pricytological, during which acantholytic cells are detected in fingerprint smears.

Histological. With its help, the 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 all possible diseases, according to the clinical manifestation of the disease.

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

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

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

Blood test
The platelet count determines the general state of health and immunity.
Urinalysis
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.

Treatment

Glucocorticoids (prednisolone) are the drug of choice, without which patients die. Complex regimens are used depending on the severity of symptoms. When a positive effect is achieved, the dose is reduced very slowly. They try to achieve a minimum at which new bubbles do not appear - it is taken on an ongoing basis.
Cytostatic drugs (azathioprine, cyclophosphamide) are combined with glucocorticoids in order to prescribe less of the latter, which reduces the likelihood and severity of side effects.
Antibiotics and antifungals are used when a secondary infection occurs. They are prescribed depending on what flora is detected.
Ointments with corticosteroids and antiseptic solutions for mouth rinsing are used locally.

Plasmapheresis allows you to clear the body of autoantibodies and can be used to prevent relapses. At the same time, you should monitor blood pressure, blood sugar levels, and regularly check internal organs, since glucocorticoids and cytostatics, unfortunately, are highly likely to provoke complications.

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Treatment for pemphigus begins after diagnosis. This is done on the basis of 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 itch, antihistamines are prescribed: 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 should not be used by children!
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: an aqueous solution of 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.

Hormonal therapy often causes complications, such as obesity, diabetes, gastric ulcers, hypertension, thromboembolism, pancreatitis and decreased immunity. Therefore, in order to avoid the occurrence of concomitant diseases, you should follow a special diet rich in vitamins and protein, limit the consumption of salt and carbohydrates.

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

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

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.

True pemphigus, which is an autoimmune disease, can be treated with hormonal drugs.

Important. Since the disease is fatal without proper therapy, there are no contraindications to the use of corticosteroids in this case. The benefits of their use far outweigh the risk of side effects.

The main drugs used in the treatment of pemphigus are Prednisolone and Dexamethasone. They start taking them with high doses, then there is a gradual decrease to a maintenance dose - one at which the appearance of fresh rashes will not be observed.

Cytostatic agents are prescribed simultaneously with corticosteroids. Usually this is Methotrexate or Cyclosporine. A long-acting corticosteroid is also used - Diprospan in injections.

Local treatment of pemphigus in adults is of secondary importance. Bubbles and erosions are treated with antiseptics - brilliant green solution, fucorcin. For extensive rashes, baths with potassium permanganate are used.

Solcoseryl, a paste with a regenerating effect, is applied to the mucous membranes. Apply rinses with antiseptics. Thorough sanitation of the oral cavity must be ensured.

Treatment of dermatitis herpetiformis involves the use of DDS - diamine diphenyl sulfone. The drug is taken in courses. A diet excluding gluten products is indicated. Local treatment is the same as for true pemphigus.

Treatment of viral pemphigus in adults involves the use of antiviral drugs - orally and locally. The drug is determined by the doctor after examining the contents of the blisters.

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 a 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.

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.

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 the immune system.

To strengthen it and prevent the disease, smoked and fatty foods should be excluded from the diet. Walking in the fresh air and doing physical exercise are remarkably helpful in reducing the risk of relapse.

Folk remedies for pemphigus

Thanks to alternative medicine, it is impossible to get rid of such a dermatological disease as pemphigus, but it can actually alleviate the condition of 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 aloe leaves, 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;
Pour 2 tablespoons of chopped meadow clover flower heads into 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, going outside; monitor your immunity, avoid overwork; respond promptly to signs of any disease and do not neglect the full course of treatment; adhere to a healthy diet and active lifestyle.

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 a mild course, ending with recovery;
encephalitis - inflammation of the brain. It develops rarely and can occur in varying degrees of severity;
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 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

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 a person with viral pemphigus, in order to reduce the chance that you will get sick, you need to eat nutritiously for the next week, enriching your diet with a sufficient amount of vitamin foods (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, take 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;
blood pressure control;
control over prothrombin.

Pemphigus vulgaris in the oral cavity

People who have been diagnosed with pemphigus should be regularly monitored by a dermatologist. All information about the course of the disease and methods of its treatment are reflected in the patient’s medical history.

Those who have recovered from pemphigus are recommended to work with a gentle work schedule. You should also be careful about physical activity and avoid exposing the skin surface to sunlight. It is necessary to change underwear and bed linen frequently.

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 of drug therapy. As well as correction of the child’s nutrition and lifestyle.

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Pemphigus is a dermatological disease manifested by a blistering rash on the skin. In severe cases, a secondary infection may occur. What causes this disease in an adult, what types are there and what is used for treatment?

What kind of disease is this

The term “pemphigus” combines a number of skin diseases with similar symptoms. They can be caused by various reasons. The main symptom for which this group was collected is a blistering rash on the skin. In some cases, the rash also affects the mucous membranes.

Varieties

The group of blistering dermatoses includes the following diseases:

pemphigus classic or pemphigus;

pemphigus ocular or cicatricial pemphigoid;

bullous pemphigoid;

Dühring's dermatitis herpetiformis.

The classification is based on the mechanism of formation of pemphigus vesicles. True pemphigus is pemphigus. Rashes with it are formed as a result of acantholysis - destruction of the spinous layer of the epidermis. The disease is serious and can be fatal.

All other types are pemphigoid, or non-acantholytic pemphigus in adults. It is less severe and usually ends favorably.

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Reasons for appearance

It is more convenient to consider the causes of pemphigus by individual diseases. In some cases the reasons are similar, in others they are different.

Classic pemphigus has been proven to be an autoimmune disease.
Pemphigus of the eyes is also of autoimmune origin.
Bullous pemphigoid is a disease of autoimmune origin. It may occur as a manifestation of paraneoplastic syndrome in tumor diseases.
Dermatitis herpetiformis or Dühring's disease is a disease of unknown etiology.

Note. It is believed that there is a hereditary predisposition to Dühring's disease, and gluten intolerance is important. There is also a theory that it is viral pemphigus in adults.

Symptoms

Despite the common symptom in the form of a blistering rash, different diseases have their own, characteristic only for them, manifestations.

Pemphigus

The first rashes appear on the mucous membrane of the oral cavity, in the pharynx. Gradually the rash spreads to the entire body. Pemphigus vesicles on the mucous membrane are very thin and burst easily. In their place, erosive changes form. As a result, eating and even talking are very difficult due to pain.

Note. The disease can have a benign course, in which the patient’s condition practically does not worsen. There is also a malignant course with severe intoxication, exhaustion and dehydration.

According to the characteristics of symptoms, four forms are distinguished:

pemphigus vulgaris;

pemphigus foliaceus;

seborrheic pemphigus, or erythematous;

vegetative.

With pemphigus vegetans, the rash tends to be located in skin folds, around the natural openings of the body and the navel. After the blisters open, instead of erosions, skin growths appear - vegetations. They have a grayish color. Papillomas can merge and form extensive lesions. The growths produce abundant fluid. Patients experience severe pain and itching.

The leaf-shaped form of pemphigus is often found in children. Bubbles with this option consist of several layers of the epidermis. After opening them, scaly crusts form on the skin.

Important. This disease lasts for many years, gradually spreading to the entire skin, including the scalp. The larger the lesions, the worse the patient's condition.

A type of pemphigus foliaceus is Brazilian or epidemic pemphigus. It often affects all members of the same family. The disease is widespread in South America. There is a high probability that this pemphigus is infectious, but its causative agent has not yet been identified.

Seborrheic pemphigus is called Senir-Usher syndrome. By its origin, this is true pemphigus - it can develop into other variants of pemphigus. The main part of the rashes is localized on the skin. If bubbles appear on the mucous membrane, this is an unfavorable sign. The blisters are practically invisible; they immediately become covered with yellow crusts, as with seborrhea.

Pemphigus eye

The disease is typical for women over 50 years of age. First, conjunctivitis develops - unilateral or bilateral. Then, against the background of the inflamed conjunctiva, thin blisters form. After opening them, adhesions are formed, which lead to the fusion of the eyelids with each other. The eyeball becomes motionless and blindness develops.

Note. In addition to the conjunctiva, pemphigus vesicles appear on the oral mucosa. There they are dense and tense. After opening them, deep painful erosions form.

Bullous pemphigoid

Bubbles appear on symmetrical areas of the body - on the sides of the torso, inner thighs. The background may be unchanged or hyperemic skin. Some blisters contain hemorrhagic contents. The rash is accompanied by itching.

The blisters can merge and reach several centimeters in diameter, forming bullae.

Dühring's dermatitis

Occurs at puberty, more often in men. Unlike other types of pemphigus in adults, the rash here is polymorphic. First, hyperemia of the skin appears, on which blisters, papules and blisters form. After some time, erosions and crusts form in their place. The rashes resemble those of herpes - they are located in separate groups, on symmetrical areas of the body. Accompanied by severe skin itching.

Diagnostics

The diagnosis is based on a characteristic clinical picture. Confirmation is a histological examination of the epidermis. If acantholysis is detected, the diagnosis of pemphigus is considered reliable.

Pemphigoids - bullous and cicatricial - are diagnosed based on the characteristic clinical picture. Histological examination of the epidermis does not reveal acantholysis of the stratum spinosum.

Dühring's dermatitis is diagnosed based on the typical clinical picture. Of the specific methods, the Jadassohn test is used - when applying an iodine solution to the skin, the number of rashes increases.

Treatment

True pemphigus, which is an autoimmune disease, is subject to treatment with hormonal drugs.

Important. Since the disease is fatal without proper therapy, there are no contraindications to the use of corticosteroids in this case. The benefits of their use far outweigh the risk of side effects.

The main drugs used in the treatment of pemphigus are Prednisolone and Dexamethasone. They begin taking them with high doses, then there is a gradual reduction to a maintenance dose - one at which the appearance of fresh rashes will not be observed.

Cytostatic agents are prescribed simultaneously with corticosteroids. Usually this is Methotrexate or Cyclosporine. A long-acting corticosteroid is also used - Diprospan injection.

Local treatment of pemphigus in adults is of secondary importance. Bubbles and erosions are treated with antiseptics - brilliant green solution, fucorcin. For extensive rashes, baths with potassium permanganate are used.

Solcoseryl, a paste with a regenerating effect, is applied to the mucous membranes. Apply rinses with antiseptics. Thorough sanitation of the oral cavity must be ensured.

Treatment of dermatitis herpetiformis involves the use of DDS - diamindiphenyl sulfone. The drug is taken in courses. A diet excluding gluten products is indicated. Local treatment is the same as for true pemphigus.

Treatment of viral pemphigus in adults involves the use of antiviral drugs - orally and locally. The drug is determined by the doctor after examining the contents of the blisters.

Forecast

Some cases of pemphigus in adults end favorably. Some have a tendency to become chronic and last for many years. Some cases without adequate treatment lead to the death of the patient. Death occurs due to extensive damage to the skin, secondary infection and the development of sepsis.

Discussion

Pemphigus belongs to a group of potentially fatal dermatoses that affect the skin and mucous membranes. Clinically, it is manifested by the appearance of vesicles or blisters, which are the first to be diagnosed in the oral cavity and precede the symptoms of skin lesions. In some cases, oral vesicles may even occur several months before signs of pemphigus on the body become clinically diagnosable. That is why symptoms of damage to the oral mucosa, given their primary nature, can help in making the correct diagnosis and providing timely and adequate treatment, which is extremely important for reducing the risk of adverse consequences in such a dangerous disease as pemphigus. This article presents a clinical case of pemphigus vulgaris, the first symptoms of which were found in the tongue area, which further confirms the importance of timely diagnosis of oral cavity pathologies in the prevention and treatment of general somatic diseases.

Pemphigus vulgaris is the most common disease among the Pemphigus group of pathologies, which are potentially fatal autoimmune lesions of the skin and mucous membranes. As a rule, it is characterized by the appearance of blisters in the oral cavity, which in 50% of cases are primary and precede clinically diagnosed signs of the disease in the skin area. The peak incidence occurs between 40 and 50 years of age. Clinically, cavity lesions in most cases appear in the form of blisters, which quickly burst, forming painful erosions. The mucous membranes of the cheeks, lips and soft palate are often affected. The diagnosis is made on the basis of verified clinical manifestations and is confirmed by studying the biopsy material. To definitively confirm the diagnosis of pemphigus vulgaris, an immunofluorescence method for identifying antibodies is used. Given the primary nature of lesions in the tissues of the oral cavity, the dentist has a huge responsibility for the verification of such lesions, the registration of which at an early stage ensures the effectiveness of therapeutic manipulations in the future. This article presents a clinical case of pemphigus vulgaris with a primary lesion of the surface of the tongue, the early diagnosis of which helped to form an optimal algorithm for effective treatment and preventive measures in the future, thereby minimizing the risk of an unfavorable outcome.

Clinical case of pemphigus vulgaris with damage to oral tissues and without clinical signs of pathology in the skin area

A 55-year-old man presented to the dental clinic with painful, non-healing ulcers on the posterolateral surface of the tongue and buccal mucosa. Based on the anamnesis, it was found that these lesions provoke in the patient feelings of discomfort and burning that have not gone away over the past six months. The patient confirmed that some time ago a blister formed on the cheek, which quickly burst, after which ulcerative lesions appeared in the area of the cheek and tongue. In the cheek area, the ulcer had an ovoid shape measuring 2 cm × 2 cm with beveled edges along the line of occlusion from teeth 35 to 37 (photo 1). On the tongue, the lesion was smaller, measuring 1 cm × 1 cm, also oval in shape, covered with a yellow crust (Figure 2). Having made sure that there were no traumatic agents such as sharp dental edges, cusps or inadequate boundaries of the prosthesis, a preliminary diagnosis of vesiculobullous lesions such as pemphigus, pemphigoid, or the bullous form of lichen planus was made. After an incisional biopsy, biopsy specimens of the affected tissues were generated and subject to further histological examination. Biopsy material was also formed from the area of tissue adjacent to the pathology site; it was later sent for additional immunofluorescent analysis. The results of histopathological examination of both pathological areas were identical: the structure of the biopsy specimens revealed ulcerated stratified squamous epithelium with traces of detachment of the basal layer (photo 3) and round acantholytic Tzanck cells with hyperchromic nuclei (photo 4). Below the areas of detachment, basal cells were attached to the connective tissue structure, in which a dense inflammatory infiltrate of plasma cells was observed. This picture was morphologically reminiscent of pemphigus vulgaris, which was ultimately confirmed by the results of immunofluorescence analysis and verification of IgG and C3 (complement) depots along the spinous intercellular region.

Photo 1: Ovoid-shaped ulcer in the mucous membrane of the left cheek.

Figure 2: Yellow crusted ulcer on the lateral posterior surface of the tongue.

Photo 3: Suprabasal detachment in the epithelial structure (hematoxylin-eosin staining, × 100).

Photo 4: Acantholytic Tzanck cells in the area of suprabasal detachment (hematoxylin-eosin staining, × 400).

Discussion

The name of the pathology described above comes from the Greek word meaning “blister”, and the term covers a range of potentially fatal autoimmune lesions of the skin and mucous membranes in which intraepithelial bullae form. Bubbles arise in the epithelium as a result of an immune reaction of IgG autoantibodies in response to the appearance of the structural proteins of desmosomes - desmogleins 1 and 3. Recently, a significant role in the development of pathology has been assigned to the antigen desmoglein 4 and other antigens of non-desmoglein nature like the human alpha-9-acetylcholine receptor , which regulates the adhesion of keratinocytes and the binding of keratinocyte-like derivatives (pempgakisna and catenin). When the interaction of these substances is disrupted, the process of acantholysis and detachment of the suprabasal layer occurs.

Pemphigus typically affects patients between 40 and 50 years of age and is more common in women than men. In more than 50% of cases, in patients with pemphigus, initial manifestations were found on the oral mucosa with subsequent involvement of the skin in the pathological process. The average duration of oral symptoms before the onset of other signs of disease ranged from 3 months to one year.

In the clinical case described above, the patient sought help for lesions of the oral mucosa, which were preceded by the appearance of a bubble in the cheek area. After the bladder ruptured, a painful erosion formed in its place, which bothered the patient for four months. No lesions were found on the skin, but the presence of pathology on the oral mucosa with characteristic pain and burning is already a full-fledged argument for making a preliminary diagnosis of pemphigus vulgaris. According to the literature, this pathology in most cases begins to manifest itself with dental symptoms, and only then migrates to the skin area. It is interesting that lesions of the tongue in pemphigus vulgaris are a rare manifestation, but in our case it was this, as well as the presence of lesions in the cheek area, that became the first signs of pathology. Because the clinical manifestations of pemphigus vulgaris are similar in nature to those of cicatricial pemphigoid and bullous lichen planus, the diagnosis should always be confirmed by histopathology and immunofluorescence analysis. After examining the biopsy specimens, signs of suprabasal dissection and acantholytic Tzanck cells, which are formed due to the formation of intraepithelial bullae, were found in their composition. Immunofluorescence analysis revealed a typical pattern of deposition of IgG and complement C3 in the form of a fishnet among the structure of the spinous layer, as a manifestation of an autoimmune reaction in pemphigus vulgaris. Thus, both histopathological and immunofluorescence studies confirmed the diagnosis of Pemphigus vulgaris.

Treatment of pemphigus vulgaris is usually carried out through local and general administration of corticosteroid drugs. The essence of therapy comes down to systemic immunosuppression through the use of corticosteroids and adjuvant drugs, such as methotrexate, cyclophosphamide, etc. Cholinergic drugs, in turn, help stop the process of acantholysis in the epithelial structure. Our patient, after an individual consultation in the dermatology department, was prescribed 100 mg of dexamethasone for 3 days along with 500 mg of cyclophosphamide. This course is planned to be extended for two more cycles with an interval of 4 weeks. During the 4-week period, the patient was prescribed 30 mg of Visolin in tablet form. After 2 weeks of steroid therapy, healing of the affected areas of the cheeks and tongue was found, indicating a positive result of therapy.

Since pemphigus vulgaris is a fatal disease, early diagnosis of its first symptoms on the oral mucosa is important for effective prevention of future complications. The diagnosis of pemphigus is based on the recording of clinical signs, as well as on the results of histopathological and immunofluorescence studies. But in most cases, only the clinical signs of damage to the oral mucosa are enough to suspect this pathology. This approach, in turn, facilitates timely treatment, which helps to avoid most undesirable and even fatal consequences. However, regular long-term monitoring is equally important to identify possible signs of relapse of the disease, which is extremely important to stop in time.

Contents of the article

Etiology unknown. Various concepts have been proposed: endocrine disorders (adrenal insufficiency), metabolic (water) disorders, viral damage, immune disorders. The most accepted classification comes down to the following division:
1. Pemphigus vera with the formation of acantholytic cells: pemphigus vulgaris (vulgar), vegetative, foliate, seborrheic (or erythematous).
2. Benign pemphigus - non-acantholytic (in the absence of acantholytic cells): actually non-acantholytic (Lever's bullous pemphigoid), pemphigus of the eye, benign non-acantholytic pemphigus of the oral mucosa only.

Pemphigus Clinic

The clinical picture is quite typical. The onset of the disease is characterized by the appearance of whitish spots on the unchanged mucous membrane of the oral cavity or on the skin (usually the back), on the basis of which single or multiple blisters soon develop. The latter quickly burst, forming an erosive surface with a bright bottom without fibrinous plaque, which tends to merge with neighboring elements. Erosions do not bleed and are localized in the distal parts of the oral mucosa. The tongue is swollen, with traces of teeth marks. Hypersalivation, bad breath, and hoarse voice are noted. For all types of pemphigus, the most typical is Nikolsky's symptom - when the bubble is pulled with tweezers, a section of skin or mucous membrane peels off over a long distance from the bubble. When rubbing an area of skin or mucous membrane next to the bubble, when lifting the skin with an instrument, the area of skin or mucous membrane also splits off. The process is accompanied by symptoms of intoxication and immunodeficiency. Often bubbles appear in the area of the eye, nose, esophagus, and genitals. The process occurs with relapses. To clarify the diagnosis, cytological studies are carried out: the presence of acantholytic cells is characteristic of pemphigus. Benign pemphigus occurs more calmly. General symptoms are mild, Nikolsky's sign is absent, acantholytic cells are not detected.

Treatment of pemphigus

Corticosteroid therapy (cortisone, prednisolone, triamcinolone). Treatment begins with the use of prednisolone
2 tablets (5 mg) 6-8 times a day, the daily dose should not exceed 80 mg. If there is an effect after a month, the administration of steroids can be gradually reduced to a maintenance dose (1 tablet 3 times a day). Steroid drugs can be discontinued only when the typical symptoms completely disappear. General restorative therapy is prescribed: transfusion of plasma, blood substitutes, multivitamins, amino acids. For local treatment, antiseptic agents are used - lotions, rinses. It is advisable to lubricate the affected areas with hydrocortisone emulsion, rosehip oil, sea buckthorn oil, and locacorten. In severe cases of the disease, especially when complications occur, broad-spectrum antibiotics are prescribed. Patients with pemphigus should be treated in a hospital setting and subsequently undergo clinical observation.

A group of rare, but often very severe, disabling, and sometimes deadly vesiculobullous (i.e., blistering) autoimmune diseases, the spread of which affects the skin and mucous membranes.

Causes of pemphigus have not been established to date, but there are a number of considerations in this regard. According to most studies, the main role in the pathogenesis of this disease belongs to autoimmune processes, as evidenced by:

formation of antibodies to the intercellular substance;
fixation of the antigen-antibody complex in the intercellular substance, which is believed to cause the destruction of desmosomes of epidermocytes or mucosal epithelium;
loss of the ability of cells to connect with each other, the development of acantholysis, although its mechanism is complex and not fully understood.

Pemphigus most often develops in women aged 40-60 years, although it can occur at any age (however, it is rare in children).

Clinical manifestations of pemphigus are characterized by the causeless development of flaccid or tense bullous elements on unchanged skin or mucous membranes. More often these are single bullous elements on the mucous membranes of the mouth, in the area of natural folds, on the scalp, and torso. The surface of these elements quickly collapses, and the contents dry out, forming crusts; for a long time, the disease can be hidden under the mask of impetigo.

In other cases, according to patients, “the skin seems to float” and the erosions do not crust over. According to summary data, the onset of the disease with the formation of erosions on the oral mucosa is observed in 85% of cases (here they do not heal for a long time, even under the influence of anti-inflammatory therapy), and dissemination of the rash on the skin occurs after 1-9 months. Less commonly, the disease begins with damage to the mucous membranes of the genital organs and larynx. Sometimes only damage to the red border of the lips is observed for a long time. On the eve of the dissemination of the process, patients may experience malaise, increased body temperature, and anxiety.

The rash is monomorphic in the form of bullous elements on any part of the skin, their content is serous, then cloudy and purulent. The size of the rash elements ranges from several millimeters to several centimeters; they tend to grow peripherally and form scalloped lesions. Bullous elements are destroyed at the slightest injury, forming red juicy erosions, along the periphery of which there are shreds of tires. During this period of the disease, Nikolsky’s symptom is always positive (when tweezers are pulled by the scraps of crusts towards healthy skin, the epidermis exfoliates outside the bullous element by several millimeters in the form of a ribbon; the second version of Nikolsky’s symptom is that with intense rubbing of healthy-looking skin with a finger lesions, less often in distant areas, the epidermis peels off, leaving a moist surface). The severity and severity of the pathological process in pemphigus is determined not by inflammatory phenomena, but by the development of fresh bullous elements. In recent years, some pathomorphism of the disease has been observed - bullous elements appear on an erythematous, edematous basis, and there is a tendency to group (“pemphigus herpetiformis”).

The classification of pemphigus is represented by the following varieties:

herpetiformis,
vegetative,
leaf-shaped,
erythematous,
caused by taking medications.

For herpetiformispemphigus characteristic:

herpetiform rash, accompanied by burning and itching;
suprabasal and subcorneal acantholysis with the formation of intraepidermal bullous elements;
deposition of immunoglobulins B in the intercellular space of the epidermis.

A characteristic clinical feature of pemphigus is the very slow epithelization of erosions. In folds, due to the friction of erosive surfaces, granulation or even vegetation may develop. Pigmentation remains in places where the rash develops back.
Most often, without treatment, the process constantly progresses. Sometimes, with a “malignant” course, a rapid generalization of the rash is observed with damage to the mucous membranes, a severe general condition due to intoxication, edema, fever, and after a few months death occurs. Early generalization of the process portends a poor prognosis.

In other cases, local damage or damage only to the oral mucosa is observed, with a long course without disturbing the general condition and significant generalization of the process. With adequate corticosteroid therapy, in most cases the process stops, the erosions epithelialize and it seems that a complete recovery has occurred. But patients need long-term, often lifelong, maintenance therapy.

Early histological changes are intracellular edema and the disappearance of intercellular bridges in the lower third of the osteo-like layer (acantholysis); as a result of acantholysis, fissures are formed first, and then bullous elements, the basal cells lose connection with each other, but remain attached to the basement membrane, round keratinocytes - acantholytic cells - are detected in the bullous elements.

Clinic pemphigus vegetans It is represented by bullous elements, often first appearing on the oral mucosa, especially at the places where it passes into the skin. At the same time or a little later, a similar rash appears on the skin around natural openings and in skin folds. Bullous elements quickly collapse, forming bright red erosions and tend to grow peripherally. On the surface of these erosions, in the next 6-7 days, succulent, first small, then large, bright red vegetation with discharge and an unpleasant odor appear. Merging, the lesions form vegetative plaques with a diameter of 5-10 cm of various shapes, on the periphery of which long-lasting pustules are sometimes observed.

Nikolsky's symptom is positive directly at the lesions. Acantholytic cells can also be found on the surface of plaques. The course of pemphigus vegetans is long, sometimes quite long remissions are observed; it is possible to transform ordinary pemphigus into vegetative pemphigus, and vice versa.

Clinic pemphigus foliaceus in the initial stages it may resemble erythema-squamous changes in exudative psoriasis, eczema, impetigo, seborrheic dermatitis and the like. Sometimes, first, superficial, flabby bullous elements with a thin lid appear on unchanged or slightly hyperemic skin; they quickly collapse, forming juicy red erosions, on the surface of which the exudate dries into layer-by-layer scale-crusts and superficial bullous elements form again under them. In some cases, the cavity elements are small and located on an edematous, erythematous base, which resembles Dühring's dermatitis herpetiformis. Subsequently, as a result of peripheral growth, significant erosive surfaces are formed, partially covered with crusts resembling exfoliative erythroderma.

Nikolsky's symptom is well expressed near the lesions and in distant areas. Acantholytic cells are found in fingerprint smears. In cases of prolonged course, limited foci with pronounced follicular hyperkeratosis are formed in certain areas of the skin (face, back), which, according to some researchers, is pathognomonic for pemphigus foliaceus. The mucous membranes are not involved in the pathological process.

When the process generalizes, the general condition is disrupted, body temperature rises, a secondary infection occurs, cachexia develops, and patients die.

Pathohistological changes are characterized by the presence of intraepidermal fissures and bullous elements, which are localized under the granular or stratum corneum of the epidermis; pronounced acantholysis; in old lesions - hyperkeratosis, dyskeratosis of granular cells. During the diagnostic process, attention is paid to the presence of flaccid bullous elements, lamellar peeling, reappearance of bullous elements in previous erosive-crustal areas and other symptoms characteristic of pemphigus.

Clinic erythematous pemphigus consists of individual symptoms of lupus erythematosus, pemphigus and seborrheic dermatitis. Most often it is localized on the skin of the face (in the form of a butterfly), the scalp and, less commonly, the body (the area of the sternum and between the shoulder blades). Erythema lesions appear with clear boundaries and thin, fluffy gray crusty scales on the surface. The lesions are often moist, weeping, then gray-yellow or brown crusts form on the surface as a result of the drying of the exudate of flaccid bullous elements that form on these lesions or neighboring areas and are very quickly destroyed. Lesions on the face can exist for months and years, and only then does generalization occur. On the scalp, the rash has the character of seborrheic dermatitis, but there may also be limited lesions with dense massive crusts and exudate. In these places, atrophy and alopecia may develop. Sometimes, near erythema-squamous foci, single small flabby thin-walled bullous elements can be observed.

Nikolsky's sign in the affected areas is positive. In a third of patients, damage to the mucous membranes is possible. The course is long, with remissions. The process may deteriorate after ultraviolet irradiation.

Pemphigus caused by medication does not differ from normal pemphigus in its clinical picture, cytological and immunological parameters. When the effect of certain drugs is eliminated, a favorable prognosis is possible. The following medications can cause the development of pemphigus:

D-penicillamine (cuprenil),
ampicillin,
penicillin,
captopril,
griseofulvin,
isoniazid,
ethambutol,
sulfonamides.

This happens very rarely and in most cases the rash disappears after stopping these medications.
All patients with various clinical forms of pemphigus are assigned a disability group depending on the severity of the disease, and they are forced to take maintenance doses of corticosteroids throughout their lives.

How to treat pemphigus?

Main in treatmentpemphigus is the use of glucocorticosteroid hormones, all other medications are of auxiliary value.

The general principles for the use of these hormones are:

initial loading doses for stabilization and regression of the rash;
gradual dose reduction;
individual maintenance doses, in most cases throughout life.

There is no consensus on initial loading doses. Some experts believe that in the case of active generalization of the process, 150-180 to 360 mg of prednisolone per day should be prescribed, while others recommend 60-80-100 mg/day and only if this dose does not produce an effect for 6-7 days, it should be doubled. There are methods according to which 150-200 mg of prednisolone per day is prescribed for 4-6 days, then the dose is reduced to 60 mg or half, and this dose is again used for a week, followed by a reduction of 50%, and then the dose is reduced gradually.

The administration of 1 g of methylprednisolone sodium succinate for 3 days (pulse therapy) was effective when this dose was administered over 15 minutes and in subsequent days was reduced to 150 mg per day.

The question of the duration of use of maximum (loading) doses of corticosteroids and tactics for reducing them is important. Most authors are of the opinion that the maximum daily dose should be maintained until a pronounced therapeutic effect and epithelization of erosions occur.

One of the options for reducing the maximum dose is as follows: during the first week, the dose is reduced by 40 mg, the second - by 30 mg, the third - by 25 mg to a daily dose of 40 mg, the dose reduction is carried out against the background of the use of cytostatics: methotrexate (20 mg per week ), cyclophosphamide (100 mg per day) or azathioprine (150 mg per day). Against this background, the daily dose of prednisolone is reduced by 5 mg monthly, and at a dose of 15 mg per day - by 5 mg every 2 months. Please note that these are only general recommendations because each patient responds differently to corticosteroids and the rate at which they are tapered.

It should be noted that erosions on the oral mucosa epithelialize very slowly and therefore it is not worth continuing treatment with high doses of corticosteroids.

The form of administration of steroids is also of practical importance. One of the options is this: with an active disseminated process, 60 mg of prednisolone (12 tablets) is prescribed orally, taking into account the daily biorhythm of the release of steroids into the blood, and 60 mg of prednisolone (2 ampoules of 30 mg each) is prescribed intramuscularly. In the process of reducing the daily dose, first of all, the injection form is canceled (30 mg - 1 ml per week).

It should be noted that in some cases the process is resistant to steroids and, in general, to individual drugs. In this case, prednisolone can be replaced with triamcinolone, methylprednisolone, dexamethasone, betamethasone in equivalent doses.

It should be noted that when treatmentpemphigus There are practically no contraindications for the administration of corticosteroids, since without their administration the disease is fatal.

In order to reduce the dose of corticosteroids, in addition to their combination with cytostatics, heparin, plasmapheresis, hemosorption, and proteinase inhibitors (contrical) are used simultaneously. Injections of gammaglobulin, interferon, riboxin, vitamins, blood transfusion, plasma, diphenyl sulfone are indicated.

Sometimes, in addition to steroids, riboflavin or benzaflavin is recommended for the treatment of erythematous pemphigus.

Maintenance therapy, selected individually for each patient, must be carried out permanently for years. Apart from clinical ones, there are no other objective criteria for monitoring steroid dose reduction.

If pemphigus recurs, the maintenance dose is doubled and, if necessary, increased further. When erosions are localized on the oral mucosa, doxycycline, methotrexate, nizoral, dipheny are periodically indicated; in case of complications with candidiasis - nizoral and fluconazole, pyoderma - antibiotics, steroid diabetes - antidiabetic drugs after consultation with an endocrinologist.

External therapy for pemphigus is of secondary importance. Aerosols with corticosteroids and antibiotics (oxycyclosol, oxycort, polcortolone), corticosteroid creams, fucorcin, xeroform, syntomycin liniment are used. When the process is localized in the mouth, frequent rinsing with a solution of soda, boric acid with the addition of a 0.5% novocaine solution is recommended. Insolation is strictly contraindicated for patients with pemphigus.

The prognosis is difficult both for life and for recovery. Only in a few patients after long-term therapy is it possible to completely cancel GCS. Life is threatened by the disease itself and its complications, as well as long-term exposure to corticosteroids. Depending on the condition, such patients are transferred to the appropriate disability group. Patients die from complications: pneumonia, sepsis, cardiovascular failure, cachexia, etc.

Prevention of pemphigus has not been developed.

What diseases can it be associated with?

The development of pemphigus is often accompanied by complications, especially against the background of absent or inadequate treatment. However, therapy appropriate to the diagnosis can affect health over the years, since it is usually lifelong use of corticosteroids.

Complications of pemphigus are:

These often cause death.

Treatment of pemphigus at home

Treatment of pemphigus mainly occurs at home, hospitalization is necessary in acute and critical conditions, in the presence of complications or at the stage of forming a treatment regimen. It is contraindicated to self-medicate at home; it is important to follow doctor’s prescriptions exactly.

What medications are used to treat pemphigus?

Treatment of pemphigus usually carried out with hormonal drugs, which are taken in loading doses, and then their concentration is sought to be reduced to a minimum; it is extremely rare that it is possible to completely stop taking the drugs. Specific medication regimens are determined by the attending physician in each individual case, focusing at least on the individual tolerance of the prescribed regimen by the patient.

The following pharmaceuticals are used:

- 150 mg per day,
- 10,000 units 2 times a day intramuscularly for 15 days,
- 0.1 g 2 times a day,
- 0.1 g 2 times a day,
- 20 mg per week,
- from 40 to 180 mg per day,
- 100 mg per day.

Treatment of pemphigus with traditional methods

Pemphigus is a disease prone to frequent relapses, the treatment of which mainly lasts for life. It is never too late to resort to folk remedies for this disease, but it is better to discuss the choice of such with your doctor. You can take note of the following recipes:

combine chopped onions and garlic, salt, black pepper and honey in equal proportions, place in a preheated oven and simmer there for 15 minutes; use the resulting viscous slurry for applications on exposed bullous elements, which will help draw pus out of them and speed up healing;
Grind 80 grams of fresh walnut leaves and pour in 300 ml of vegetable oil (olive, sunflower, corn or any other), leave in the dark, but at room temperature for 21 days, shake periodically; Strain the resulting oil and use it to lubricate opened lesions;
2 tbsp. place meadow clover inflorescences in a thermos, pour a glass of boiling water, leave for 2 hours, strain; use for washing erosions due to pemphigus.

Treatment of pemphigus during pregnancy

Due to weakened immunity and hormonal changes, pregnant women have a slightly higher risk of encountering pemphigus. In addition, they separately consider the so-called pemphigus of pregnancy - an irritation that grows from the navel over the abdomen, back, buttocks, somewhat similar to herpes, but is not one.

With the development of pemphigus in pregnant women, the risk of premature birth slightly increases, while at the same time, the statistics of miscarriages and stillbirths are still being assessed. Every twentieth child from a woman with pemphigus experiences irritation after birth.

It is important to carry out treatment of pemphigus in pregnant women exclusively together with specialized specialists, whose competence lies in the competent selection of the safest steroid and, if necessary, antibacterial agents.

Which doctors should you contact if you have pemphigus?

Diagnosis of pemphigus is based on the following signs:

resistance to any local therapy;
frequent damage to the mucous membranes of the mouth;
positive Nikolsky sign;
identification of acantholytic cells using the Tzanck method - this study is carried out to confirm the diagnosis by identifying the so-called acantholytic cells, which are formed as a result of acantholysis (breaking the connections between cells).

The Tzanck method involves placing a glass slide on fresh erosions and acantholytic cells (imprint smear) sticking to it. A sterile gum is applied to mucous membranes with erosions, and then this surface of the gum is applied to a glass slide, thus transferring acantholytic cells onto it. Staining using the Romanovsky-Giemsa method is used.

Morphological features of acantholytic cells:

they are smaller in size than normal epidermocytes, but their nuclei are larger than those of normal cells;
the nuclei of acantholytic cells are stained more intensely;
there are always 2-3 nucleoli in the nucleus;
the cytoplasm of the cells is sharply basophilic, stained unevenly, a blue zone is observed around the nucleus, and an intense blue border is observed along the periphery.

Acantholytic cells in pemphigus often have several nuclei. However, acantholytic cells can be found in Lyell's syndrome, Darier's disease, and transient acantholytic dermatosis. These cells must be differentiated from cancer cells.

As part of the diagnosis of pemphigus, immunomorphologicalresearch by direct immunofluorescence - in 100% of cases, IgO class antibodies are detected in skin sections, which are localized in the intercellular spaces of the epidermis. The method of indirect immunofluorescence detects circulating antibodies of the IgO class against antigenic complexes of the intercellular substance of the epidermis.

Histologicalstudy reveals intraepidermal (suprabasal) bullous elements and fissures.

Differential diagnosis of pemphigus herpetiformis is carried out with bullous pemphigoid, Lyell's syndrome, dermatitis herpetiformis and other bullous dermatoses.

Differential diagnosis of pemphigus vegetans is carried out with syphilitic condylomas lata, chronic familial benign pemphigus, and pyoderma vegetans.

Differential diagnosis of pemphigus foliaceus is carried out with erythroderma, Lyell's syndrome, subcorneal Sneddon-Wilkinson pustulosis, erythematous (seborrheic) pemphigus.