Pemphigus: concept of the disease, its causes, types, methods of diagnosis, treatment and prevention. Damage to the oral mucosa: key factors in diagnosing pemphigus vulgaris Treatment of pemphigus in the oral cavity

Reading time: 7 minutes. Views 1.2k. Published 05/07/2018

Sometimes people, especially after 35-40 years of age, are diagnosed with a rare, but at the same time extremely dangerous disease - pemphigus.

Against this background, significant damage occurs to the mucous membranes and epidermis, including their deep layers. A person with such an illness needs modern treatment, otherwise there will be serious consequences for the entire body, even death.

What is pemphigus?

Pemphigus is an autoimmune disease in which extensive damage to the skin and, in some cases, mucous membranes occurs. As a result of this disease, multiple blisters are observed throughout the body.

Among the main features are:

• absence of inflammatory signs for a long time;
• the average diameter of the resulting bubbles is 1.5 – 3 centimeters;
• merging them into one huge bubble if modern treatment is not provided;
• the need for long-term therapy.

Without treatment, the outcome for the body can be disastrous; the following may develop:

• diabetes mellitus;
• sepsis;
• stomach diseases;
• stroke.

In rare cases, if there is no therapy, death occurs.

Important: In the vast majority of cases, the disease is diagnosed in people over 38 years of age. In 2% - 5% of pemphigus is observed in children from 1.5 to 10 years.

Causes

The true causes influencing the development of pemphigus have not been established. However, doctors agree that the disease is affected by:

1. Hydrobalance disorders.
2. Changes in salt metabolism.
3. Infections.
4. Disturbances in protein metabolism.
5. Acquired or congenital defects of cell membranes.
6. General weakening of the body.
7. Related chronic diseases, in particular, endocrine ones.

Important: Doctors do not rule out that the cause may be skin diseases previously suffered, for example, lichen or eczema.

Symptoms and signs

After infection, a person experiences a number of symptoms that suggest the development of pemphigus:

• Formation of blisters up to 1 centimeter in diameter in the oral cavity.
• The appearance of blisters all over the body.
• The appearance of painful erosions at the site of the lesion.
• Difficulty chewing food (if there is erosion in the mouth).
• Burning in the affected areas.
• Pain.

The main features include:

1. The formation of blisters on the skin, as well as mucous membranes, with an average size of 1 - 2 centimeters.
2. Pain when moving, eating, changing clothes, and even at rest.
3. The presence of erosions in the oral cavity and throughout the body.
4. Painful sensations on the skin.
5. Bleeding, especially in severe forms of the disease.

Note: The longer treatment is not started, the more severe the symptoms become.

Classification of the disease

Pemphigus is divided into four main types

Ordinary

Characteristic:

• affects the middle and deep layers;
• the lesion is first noted in oral cavity;
• the disease can affect all parts of the body.

Note: the ordinary type is diagnosed in 70% of cases.

Vegetative

Characteristic:

• localization in the oral cavity, nostrils, lips, groin and armpits;
• frequent bleeding in damaged areas of the skin;
• the likelihood of developing intoxication.

Note: in 60% of cases it is severe.

Leaf-shaped

Peculiarities:

• the lesion is noted on the entire surface of the dermis;
• the blisters are opening quickly;
• severe redness of individual parts of the body occurs;
• after the blisters are opened, erosions remain.

In advanced types, partial or complete baldness is diagnosed, as well as rejection of the nail plates.

Seborrheic

Peculiarities:

• more light form, in comparison with others;
• Initially, the lesion occurs in the facial area, in particular on the nose, ears, and near the eyes;
• gradual growth of blisters throughout the body;
• the appearance of painful erosions.

The first signs of seborrheic appearance are similar to lupus erythematosus.

Diagnostic methods

To confirm or refute the diagnosis, doctors carry out:

1. A thorough visual examination of the mucous membranes of the mouth and nose.
2. Examination of the upper layer of the epidermis throughout the body.
3. Palpation of existing blisters.
4. Take general analysis blood.
5. Taking a smear from the surface of erosions for cytological examination.

Note: After the cytology results, the doctor can make a diagnosis.

Which doctor should I contact?

If blisters of an unknown nature form in the mouth or on the skin, you should consult a dermatologist.

Note: in 50% of cases, dermatologists treat the patient together with infectious disease specialists, especially if the disease has progressed to severe stages, and most importantly, the person has begun to experience infectious lesions of the dermis.

Treatment of pemphigus

When pemphigus is confirmed, the doctor determines the course of treatment for the patient. Basically therapy includes:

• medications for external and internal use;
• diet;
• special skin care.

Note: Dermatologists avoid a favorable outcome if the patient does not ignore any recommendations.

Drug treatment

As a course of medication, dermatologists may prescribe:

• Anti-inflammatory ointments and tablets.
• Medicines that speed up healing.
• Hormonal remedies for the treatment of eczema and various blistering marks.
• Cytostatics.

Important: cytostatic drugs are prescribed for severe lesions, as well as when the disease has progressed to a severe form.

• Antibiotics if diagnosed infectious lesion dermis or mucosa.

Important: Each product is prescribed by dermatologists in a specific dosage.

Photochemotherapy

One of the most modern methods of treating pemphigus is photochemotherapy. It is based on irradiation of blood cells with a special apparatus.

Peculiarities:

• highest efficiency;
• acceleration of the patient’s recovery processes by 2–3 times;
• complete cleansing of the blood from dangerous and harmful substances.

Important: photochemotherapy is indicated if the patient has multiple eczemas on the body, extensive damage to the dermis, and the disease becomes severe.

Local treatment

In addition to a course of medications, local treatment is required. It is based on:

• Take a daily bath with potassium solution or oak bark decoction.

The required amount of solution or decoction per liter of water is prescribed by the treating dermatologist.

The temperature of the water in the bath should not exceed 38 - 39 degrees.

• Piercing blisters on the body with a sharp needle.

To do this you need:

• wash your hands with antibacterial or laundry soap;
• disinfect the needle with alcohol or a special product;
• carefully pierce the blister with a needle;
• then apply an antiseptic to the affected area.

• Rinse your mouth with soda solution.

For a glass of warm water, two teaspoons of soda are enough.

• Application sea ​​buckthorn oil for eczema in the oral cavity.

Important: duration and frequency local treatment determined by a dermatologist.

Diet

It is important for a person diagnosed with pemphigus proper nutrition. The daily diet should include:

Dairy products:

• curdled milk;
• cottage cheese;
• kefir.

It is better to purchase fermented milk with a short shelf life.

• Milk.
• Steamed meat dishes without spices and seasonings.
• Pumpkin and potatoes, baked or boiled.
• Raisin.
• Green apples.

Important: a person should not overeat. You should eat food 5-6 times a day.

Prevention measures

Dermatologists prescribe the following to all people with suspected pemphigus or a diagnosed disease:

1. Do not puncture the resulting bubbles with dirty objects.
2. Do not apply any alcohol-containing products to them.
3. Do not eat fried or smoked foods.
4. Do not drink alcoholic beverages in any quantity.
5. Quit smoking.
6. Get more rest and avoid physical overload.
7. Do not worry.
8. Sleep 10 hours a day, and go to bed no later than 11 pm.
9. Do not change the climate zone.

Note: It has been noted that when a person is less susceptible to stress and focuses on the positive, pemphigus is diagnosed much less frequently.

Pemphigus

In children, viral pemphigus occurs, which is transmitted by air. Children under 5–6 years of age are more susceptible to this disease.

Features of this type include:

• Triggered by enterovirus.

Before blisters form on the body, the child experiences:

• temperature more than 38.5 degrees;
• dry cough;
• diarrhea.

1. On average, minor rashes form on the palms and soles within 2 days.
2. On the 3rd day, bubbles are noted in the mouth.

Important: the blisters burst, and in their place eczema of up to 7–8 millimeters in volume is localized.

Treatment of pediatric pemphigus is determined by the pediatrician, sometimes together with a dermatologist. On average, after 7–8 days the child recovers.

Pemphigus is a serious disease, if you suspect it, you should not hesitate to visit a dermatologist. When a diagnosis is confirmed, it is important for a person to undergo treatment and fully follow the doctor’s instructions and advice. Only in this case can serious consequences, including irreversible ones, be prevented.

Blitz tips:

• do not use any traditional methods for treatment;
• do not try to buy ointments yourself and apply them to the blisters;
• never pierce them unless recommended by a dermatologist;
• strictly adhere to a special diet.

A group of rare, but often very severe, disabling, and sometimes deadly vesiculobullous (i.e., blistering) diseases 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 not determined inflammatory phenomena, but by the development of fresh bullous elements. IN last years There is some pathomorphosis of the disease - bullous elements appear on an erythematous, edematous basis, 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 vegetation of a bright red color with discharge and an unpleasant odor appears. 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 V initial stages 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 generalizing the process, it is disrupted general state, 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, according to clinical picture, cytological and immunological parameters do not differ from normal. 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 by 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 must be maintained until the onset of pronounced therapeutic effect and epithelization of erosions.

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 with a dose of 15 mg per day - by 5 mg every 2 months. It must be taken into account that this is only general recommendations, because each patient reacts differently to corticosteroids and the rate at which their dose is reduced.

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 discontinue 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 strictly follow medical prescriptions.

What medications are used to treat pemphigus?

Treatment of pemphigus usually carried out hormonal drugs, which are taken in loading doses, and then their concentration is sought to be minimized, 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's never too late to take action when dealing with this disease. folk remedies, however, 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 300 ml 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. inflorescences red clover place 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 changes hormonal levels Pregnant women have a slightly higher risk of developing 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 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.

The content 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 distal sections oral mucosa. The tongue is swollen, with traces of teeth marks. Hypersalivation is noted, bad smell from the mouth, hoarse voice. 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: 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. Used for local treatment antiseptics- 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.

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 months before body signs of pemphigus 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 cases. dangerous disease like 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 significance timely diagnosis oral 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. 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 and without damage to oral tissues clinical signs pathologies 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 for 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: an ulcerated multilayered squamous epithelium with traces of detachment of the basal layer (photo 3) and round acantholytic Tzanck cells with hyperchromatic nuclei (photo 4). Below the areas of detachment, basal cells were attached to the structure connective tissue, 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 above pathology 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 Pemphigus vulgaris is similar in nature to the features of cicatricial pemphigoid and bullous form of 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 positive result 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, helps 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.

Pemphigus (pemphigus) is a type of dermatological disease. It is quite rare and people can be susceptible to it different ages. But most often this disease is diagnosed at an older age - in age category from forty to sixty years. With pemphigus, fluid-filled blisters form on the patient’s body and mucous membranes. They cause a lot of discomfort to the patient.

Causes of the disease

The exact cause of the development of the disease is still unknown to specialists.. However, most of them believe that the disease is autoimmune in nature. It causes disturbances in functioning immune system- this leads to the fact that one’s own cells, when exposed to external factors begin to attack the body. Epidermal cells, when affected, cause disturbances between cells, and blisters form on the skin. They are filled with serous fluid, and after they open, ulcers form in their place.

All the reasons that contribute to the development of the disease have not yet been established, but experts suggest that main reason leading to the development of pathology is a hereditary factor.

It is imperative to treat pemphigus, as in severe form it can be fatal.

Pemphigus has several forms. They depend on how the pathological process manifests itself. The following main forms of pathology are distinguished:

• True pemphigus, or its acantholytic form, can manifest itself in several other varieties and is considered more dangerous and severe. It can lead to the development of serious and severe complications that can pose a threat to the health and life of the patient.
• Benign, or non-acantholytic, can also manifest itself in several forms, but is less dangerous to health and proceeds much easier.

The true form of the disease is usually divided into several types. These include:

There are also several forms of non-acantholic or benign pemphigus. One of them is the bullous form. It occurs in both children and adults. With it, blisters form on the skin, but there are no signs of acantholysis. The elements that appear may disappear without forming scars.

Sometimes benign non-acantholic pemphigus is diagnosed. With it, bubbles form only in the mouth. When examining the oral cavity, inflammation is detected.

Another type of vesicle is the scarring non-acantholic form.. It is sometimes called ocular pemphigus. It most often occurs in women after forty-five years. One of her characteristic symptoms- damage to the visual apparatus, skin and oral cavity.

Viral pemphigus is a type viral disease, which occurs in both children and adults. Moreover, the symptoms are similar regardless of the variety. One of the main features of the acantholic and non-acantholic form of the disease is its wave-like course.

If the patient is not provided with timely assistance, his condition begins to rapidly deteriorate. Common symptoms of the disease include the following:

• loss of appetite;
• appearance of weakness;
• increasing symptoms of cachexia;
• slowing down the epithelization of erosions.

Symptoms also depend on the form of the pathology. For example, with pemphigus vulgaris, blisters form on the skin different sizes. However, they have a thin-walled shell, and first appear in the mouth. When such signs are detected, the first thing a person does is consult a dentist, but a dermatologist deals with the treatment and diagnosis of such pathology. The patient may complain of pain when talking and eating and bad breath.

Symptoms may persist for 3–12 months. If treatment is not started during this time, the pathological formations will spread throughout the body. If the disease is severe, then intoxication of the body may develop, and a secondary infection may occur.

For pemphigus, the first step in treatment should be to carefully handle the blisters. Without an accurate diagnosis, it is not recommended to pierce and treat emerging elements, because this can be dangerous. You should consult a doctor as soon as possible, because the sooner treatment is started, the more effective it will be and the faster it will be possible to achieve recovery.

For treatment, ointments and creams based on glucocorticoid hormones are used. They have a powerful anti-inflammatory effect, and at first quite large doses of the drug are used. After obvious symptoms have resolved, these drugs are continued to be used to maintain the effect. If you stop using them, the disease may worsen. Sometimes, some patients are able to gradually stop taking hormonal medications.

If the form of the disease is severe, the doctor may prescribe cytostatics. These medicines are able to suppress cell proliferation and are quite effective in inflammatory autoimmune processes. When taken simultaneously with glucocorticoids, it is possible to reduce the dose of hormones and reduce their negative effects on the body.

Besides. Potassium and calcium supplements and a course of various vitamins are prescribed. And if there are infectious complications, then antibiotics are used.