True pemphigus. Causes

Among the group of bullous dermatoses, the so-called true pemphigus is distinguished. Its clinical forms include four varieties:

Ordinary, or vulgar;
vegetative;
leaf-shaped;
erythematous (Senir-Usher syndrome, seborrheic pemphigus).

The following independent nosological forms of bullous dermatoses have also been identified, in which the predominantly non-acanthomitic nature of the blisters is noted:

Bullous pemphigoid;
cicatricial pemphigoid;
chronic benign familial pemphigus Gougereau-Hailey-Hailey.

True pemphigus accounts for about 1% of all skin diseases. Women get sick more often. Average age- 40-60 years, described in children isolated cases diseases.

Etiology unknown.
Autoimmune processes play a leading role in pathogenesis.

The development of acantholysis is associated with the appearance of antibodies against the intercellular substance and the formation of an “antigen-antibody” immune complex in it. Antibodies belonging to class G immunoglobulins are detected in blood serum and cystic fluid.

The titer of circulating antibodies usually correlates with the severity of pemphigus. An integral feature true pemphigus is acantholysis with the formation of intraepidermal blisters. With pemphigus vegetans, papillomatosis, acanthosis are also observed, and in old lesions - hyperkeratosis.

Pemphigus vulgaris

Pemphigus vulgaris is the most common form of true pemphigus. It usually begins with damage to the oral mucosa and can exist in isolation for several months.
The blisters, quickly opening, form painful bright red or whitish-covered erosions, bordered by fragments of epithelium - the remnants of the bladder cover. Skin rashes appear gradually, first in the form of single blisters, mainly on the skin of the chest and back, then their number increases.

The blisters are located on externally unchanged skin, filled with transparent serous contents, and have different sizes. After existing for several days, they dry out, become covered with crusts, or open up to form bright red erosions, abundantly releasing a thick sticky exudate. Acantholysis can be detected by easy rubbing healthy-looking skin with your fingers. In this case, detachment occurs near the bubbles, and sometimes at a distance from them. surface layers epidermis with the formation of erosions (Nikolsky's symptom).
When you pull on the pieces of the bladder cover, detachment occurs. upper layers epidermis far beyond visible erosion ( marginal symptom Nikolsky).

Acantholysis also causes an increase in the area of the bladder when pressing on it with a finger (Asbo-Hansen's symptom), the acquisition of a pear shape by the bladder under the weight of exudate, and peripheral growth of erosions.

General state Few patients were affected at the onset of the disease. The pain caused by erosions is disturbing, especially severe on the mucous membrane of the mouth, which makes it difficult to eat. Gradually the condition worsens, weakness appears, low-grade fever. With the addition of pyogenic flora, the temperature can rise to higher numbers. With the progression of pemphigus vulgaris, erosions on both the mucous membranes and the skin epithelialize slowly.

Characteristic of pemphigus vulgaris is a violation of water and especially salt metabolism. Daily urinary excretion of sodium chloride is sharply reduced. Sodium chloride retention is often observed in the most early periods diseases even with isolated damage to the mucous membranes. In the absence of proper treatment, increasing cachexia leads to death. The duration of the disease (without treatment) ranges from 6 months to 2 years; in young patients, pemphigus vulgaris is more acute.

The subacute malignant variety of pemphigus vulgaris begins with damage to the oral mucosa after influenza, sore throat, tooth extraction and prosthetics.

The skin is affected after 6-12 weeks. The pathological process is characterized by the intensive growth of blisters, their fusion, and the formation of extensive erosions, along the edges of which white films hang. Nikolsky's symptom is sharply positive. The general condition is serious: intoxication, swelling and fever are noted. Without treatment, the disease is fatal within 3-6 months.

Pemphigus vegetans

At the beginning of its development, pemphigus vegetans is similar to pemphigus vulgaris. The disease often begins with damage to the oral mucosa. However, from the very beginning, blisters tend to localize around natural openings and in skin folds ( groin areas, under the mammary glands, in axillary fossae), as well as around the navel.

Subsequently, when the blisters are opened, papillomatous growths (vegetations) covered with a grayish coating develop on the surface of the erosions. Merging, they form extensive vegetative surfaces. Nikolsky's symptom is positive. Vegetations on mucous membranes are rare, but at the border with the skin (the area of the lips, nose, genitals, anus) they are common. By regressing, the vegetations shrink, flatten, and erosions epithelialize, leaving pronounced hyperpigmentation on the skin. The disease occurs chronically or subacutely (malignant); ends fatally.

Pemphigus foliaceus

Pemphigus foliaceus is characterized by a rash on the skin of superficial, flat, flaccid blisters with a thin covering, which quickly ruptures and exposes erosions or shrinks to form thin lamellar scales and crusts, layered on top of each other. Nikolsky's symptom is also pronounced on unaffected areas of the skin. Very quickly, the pathological process spreads over the entire surface of the skin and takes on the character of erythroderma with large-plate peeling. The mucous membranes are usually not affected. Hair loss and nail separation are common. The disturbances are less pronounced than with pemphigus vulgaris. The disease can last 2-5 years or more.

The general condition of patients may remain satisfactory for many months, but gradually they weaken, lose weight and, without appropriate therapy, die from increasing cachexia or from an associated infection.

Erythematous pemphigus

Erythematous pemphigus begins with the formation on the skin of the face, more often of the nose and cheeks (in the form of a butterfly), less often on the scalp, of a lesion covered with soft, easily removable yellowish scales or brownish crusts, on bottom surface which reveal soft white spines. After removing the crusts, a wet, eroded surface is revealed. Subsequently, blisters of various sizes appear on the skin of the back and chest (to a lesser extent, the limbs), quickly drying into lamellar crusts of a brownish color.

The formation of blisters can occur so imperceptibly that crusts appear as if initially, and the process resembles seborrheic eczema or impetigo. Nikolsky's symptom is positive or more often weakly positive, focal. The oral mucosa is rarely affected. The disease takes a long time and in most cases is relatively benign; sometimes there is a transition to pemphigus foliaceus or pemphigus vulgaris. Erythematous pemphigus often worsens under the influence of insolation. The onset of the disease can be triggered by a burn.

Establishing a diagnosis

The diagnosis is made on the basis of clinical manifestations and data from cytological, histological and immunological studies. To confirm the presence of acantholysis, the surface layer of cells is examined using the Tzanck method. In fingerprint smears obtained from the bottom of erosions after opening fresh blisters, pemphigus reveals acantholytic cells - spiny, differing from normal ones in their smaller size and large nucleus, which occupies almost the entire cell, heterogeneous staining of the cytoplasm (intense blue color at the periphery and light blue around the core). Polymorphism of cells and nuclei is observed; the nucleus can have from 2 to 15 large nucleoli. In subacute forms of pemphigus, multinucleated cells with deformed nuclei and “naked” nuclei without cytoplasm are found. Pemphigus is characterized by the formation of clusters of acantholytic cells in the form of layers.

Treatment of pemphigus

Treatment, regardless of the clinical form of pemphigus and the location of the rash, begins with the administration of loading doses of glucocorticosteroids. The loading dose depends on the severity of the disease. The dose is maintained after the elimination of rashes and healing of erosions for a week, and then over the course of several months it is gradually reduced to a maintenance dose. Discontinuation of treatment quickly leads to relapse of the disease, so patients must receive maintenance doses of hormones for a long time.

Corticosteroid therapy is combined with the administration of anabolic steroid drugs, potassium, calcium, and ascorbic acid. At severe course after stabilization of the process, cytostatic agents can be used.

Plasmapheresis is used to remove circulating autoantibodies from the body. Repeated procedures lead to a temporary decrease in the titer of autoantibodies and even their disappearance, which makes it possible to significantly reduce the dose of glucocorticoids and immunosuppressants.

For external treatment, warm baths with potassium permanganate are used, antibacterial ointments or aqueous solutions aniline dyes. In case of damage to the mucous membranes, washing and rinsing with a decoction of chamomile in half with a 2% solution is used boric acid, solutions of borax, furatsilin and other astringents and disinfectants.

Forecast. Highest mortality rate In patients with true pemphigus, it is observed in the first 3 years from the onset of the disease.

Pemphigus - chronic autoimmune disease, characterized by the appearance of a special type of bubbles on the surface earlier healthy skin and mucous membrane. Among the types of pemphigus can be distinguished: vulgar, vegetative, erythematous and foliate. Pemphigus can be diagnosed if acantholytic cells are detected, which are detected in a smear taken or as part of blisters in the epidermis itself (with histological examination). To treat pemphigus, glucocorticosteroids are first used (a whole course of treatment is prescribed). The latter always goes well with extracorporeal hemocorrection (plasmophoresis, cryoapherosis, hemosorption).

Causes of pemphigus

The most likely cause of pemphigus is considered to be process disturbances autoimmune system, which causes the body's cells to produce antibodies. Changes in the antigenic structure of the epidermis itself are observed due to the influence of external factors (for example, the effect of retroviruses or bad conditions the environment itself).

A negative effect on the epidermis and the production of antigens is caused by disruption of communication between cells, which is why blisters later appear. As for the risk factors for pemphigus, they have not yet been established, but it is known that people with a hereditary predisposition have a high incidence rate.

Pemphigus symptoms

Pemphigus is characterized by a long wave-like course. In the absence of proper treatment, a disturbance in the general condition of the patient may occur. In the case of the vulgar form of pemphigus, the blisters themselves will be located over the entire surface of the body, representing formations of different sizes that are filled with serous fluid. The surface of such bubbles will be thin and flaccid.

Pemphigus vulgaris

The first thing that occurs is pemphigus on the mucous membranes, so as a rule, the initial treatment will be incorrect - usually people are treated by a dentist or otolaryngologist. At this stage of the disease, patients complain of a sore throat when eating or even when talking. There is also hypersalivation and bad breath. The duration of such a symptomatic period ranges from three to four months to a year. After this, pemphigus is characterized by its spread over the surface of the body, involving everything large area skin.

Often, patients may not notice the presence of bubbles due to their small size, as well as a thin film on top. Typically, blisters can open quickly, so patients' complaints are complaints about painful erosion. Long-term and almost always unsuccessful treatment of stomatitis is often carried out. Symptoms of pemphigus are primarily blisters localized on the skin. They are distinguished by the ability to open independently, exposing an eroded surface with the remains of a tire, which often dries out and takes on the appearance of a crust.

Pemphigus in adults

Pemphigus in adults may have a bright pink color and a smooth glossy surface. Pemphigus should not be confused with erosion. Pemphigus differs from the latter in its tendency to grow peripherally and the possibility of generalization with further formation extensive lesions. If pemphigus takes a similar course, the patient’s general condition almost always worsens, intoxication begins to develop, and a secondary infection may also occur. Without rendering proper treatment patients may die.

In the case of pemphigus vulgaris, Nikolsky syndrome will be positive in the lesion. On healthy skin, in the event of even a slight mechanical impact, detachment of the upper layer of the epithelium may be observed.

Erythematous type of pemphigus

The erythematous type of pemphigus will differ from the vulgar type in the localization of skin lesions, the presence of erythematous lesions on the chest, neck and face, as well as on the scalp. The latter is seborrheic in nature. Erythematous pemphigus is characterized by clear boundaries, its surface is covered with a yellow or brown crust. If such crusts are separated from the main surface, the entire eroded surface will be exposed.

In the case of erythematous pemphigus, the blisters are usually small in size, the crust is flabby and flaccid in appearance, they often open on their own, so it is very difficult to diagnose pemphigus. Nikolsky's symptom can be localized for a long time, and in case of generalization of the process it becomes similar to a vulgar appearance.

The erythematous type of pemphigus must be differentiated from the seborrheic type of dermatitis and lupus erythematosus.

Leaf type pemphigus

The leaf-shaped type of pemphigus, in its symptoms, is a rash of the erythematous-squamous type; the blisters have thin walls and often appear on areas that were already affected. After the bubbles open, the eroded surface becomes bright red. When the surface dries out, lamellar crusts appear. Since with this form of pemphigus, blisters also form on the crusts, the affected area of the skin can become covered with a massive layer of crust due to the abundant exudate.

The leafy form of pemphigus involves the entire skin, but rarely also the mucous membranes are involved. Pemphigus leaf quickly covers the entire skin, on which blisters, erosions and fresh crusts immediately appear. Uniting with each other, the damaged areas form a large wound surface. Nikolsky syndrome will be positive even on a healthy area of skin. If pathogenic microflora attaches, sepsis will begin to develop, which is why a person’s death occurs.

Vegetating type of pemphigus

The vegetative type of pemphigus is benign. Patients throughout for long years may feel good. Bubbles are located around the holes, as well as in the area skin folds. When the bubbles open, they reveal erosions, at the bottom of which soft vegetations appear that have a fetid odor. Such vegetations are covered with serous-purulent or simply serous fluid. Pustules can be seen along the contour of the neoplasms, so pemphigus vegetans must be distinguished from chronic form pyoderma. Nikolsky syndrome will be positive only near the affected areas of the skin, however, in the terminal stages, pemphigus vegetans is similar to pemphigus vulgaris in its clinical manifestations.

Diagnosis of pemphigus

Clinic of this disease, especially in the first stages of the disease, will be uninformative, so interviewing the patient will help to exclude an erroneous diagnosis. Laboratory research will help identify pemphigus by detecting acantholytic cells when cytological examination. Histological studies will reveal the intraepidermal location of the blisters themselves.

Treatment of pemphigus

Treatment of pemphigus, first of all, consists of excluding roughage from the usual diet, simple carbohydrates, canned food, salted and salty foods. In case of damage to the oral cavity, you need to add soups and rare cereals to the diet, so as not to completely exclude food from the diet. It is known that products rich in protein content accelerate the process of cell regeneration and influence the process of epithelization of open erosions.

All patients suffering from pemphigus are monitored by a dermatologist. For such people, a lighter mode of work is indicated, stopping physical activity, avoiding sun exposure. Frequent change bed and underwear will help prevent secondary infection.

Treatment of pemphigus also involves the use of glucocorticosteroids, using them in large doses. Otherwise, positive dynamics in treatment will not be achieved. After cupping acute symptoms pemphigus, the dosage of the drugs used is reduced to the minimum level. For treatment, the method of extracorporeal hemocorrection is also used, which includes cryoapheresis, membrane plasmapheresis and hemosorption.

For local treatment of pemphigus, non-aggressive antiseptic solutions and aniline dyes are used. The prognosis of pemphigus is almost always unfavorable, since in the absence of adequate treatment, a person’s death can occur quite quickly due to complications that arise. Long-term hormone therapy significantly increases the risk of side effects, but if you refuse to take glucocorticosteroids, pemphigus will recur.

Pemphigus of newborns

Pemphigus in newborns is an acute infectious skin disease, which clinically manifests itself in the form of pustules that quickly spread throughout the entire skin.

Pemphigus in newborns is often bacterial in nature. Its causative agent is Staphylococcus aureus. Speaking about the pathogenesis of pemphigus among newborns, the skin reaction of children occupies a significant place. The skin reaction will intensify in the event of birth trauma or prematurity, as well as the incorrect lifestyle of the pregnant woman herself. Blisters will form on the child's skin due to exposure to bacterial factors.

The epidemiology of pemphigus in newborns indicates poor hygiene in the maternity hospital, the presence of chronic infections among maternity hospital staff, and the possible occurrence of autoinfected pemphigus (if a newborn develops purulent types navel diseases).

Pemphigus in newborns forms in the first days of life, but the development of the disease is possible even after one to two weeks. On healthy skin, small blisters appear with thin walls having serous contents. After a few hours, the process will generalize, the bubbles will increase in size and open. In place of the blisters, there will be painful erosions with remaining particles of the epidermis located at the edges. Such erosions will be covered with a serous-purulent crust. If pemphigus occurs in newborns, there will be intoxication, fever and lack of appetite.

If pemphigus is not cured early stages, the newborn will develop inflammatory processes internal organs (phlegmon, otitis, pneumonia). In weak newborns or premature infants, a septic form of pemphigus cannot be excluded. With the latter, the mortality rate is very high.

Pemphigus in newborns can be diagnosed based on visual inspection. Pemphigus in newborns should be distinguished from the syphilistic form of pemphigus, which is a symptom congenital syphilis. With the latter, the bubbles are located on the palms.

Antibiotic therapy can reduce the percentage of deaths from pemphigus among newborns. With timely treatment of pemphigus among newborns, the favorable outcome of the disease is significantly higher than with other types. Doctors may also prescribe the use of aniline dyes and various types of non-aggressive antiseptics.

Prevention of pemphigus is considered to be a change of underwear and bed linen, isolation of those who have pustular rashes on the skin, as well as proper monitoring of pregnant and timely provision treatment among mothers with purulent rashes.

is a disease characterized by the appearance of blisters on the skin and mucous membranes without signs of inflammation. They develop as a result acantholysis and if not applied immediately the right approach to treatment, the disease can quickly spread to the entire skin. This complication can even lead to death. This disease is more common in people over 35 years of age. Pemphigus develops equally often in people of both sexes. IN in rare cases Children can get pemphigus. According to modern research, the number of cases of pemphigus does not exceed 1.5% of all dermatoses.

Types and symptoms of pemphigus

Today, there are four types of pemphigus: ordinary, leaf-shaped, vegetative And seborrheic (erythematous). All of the above forms of pemphigus, except foliate, affect oral mucosa. The most common infection today is pemphigus vulgaris. This form of pemphigus in most cases manifests itself as rashes on the oral mucosa. And even if the first symptom of the disease is skin damage, then a little later rashes still appear on the oral mucosa. The blisters later open, and in their places appear red or covered with a white coating. erosion that surround the scraps epithelium, which are the remains of the skin of the bladder. The general condition of the patients is gradually deteriorating, patients complain of weakness, their body temperature rises. Later slows down epithelization of erosions, problems with eating appear, increases cachexia, and if the patient does not receive proper treatment, then through certain time(from six months to two years) death may occur.

On initial stages development pemphigus vegetans proceeds approximately the same as with ordinary. First, the mucous membrane in the mouth is affected. Typically, most blisters are found around natural openings in the folds of the skin. Thus, blisters can appear in the folds of the groin, in the armpit folds, under the mammary glands and in the area around the navel. Later, after the bubbles have opened, erosions appear on the surface of the resulting erosions. papillomatous growths. They are covered with a grayish coating.

With pemphigus foliaceus, special blisters appear on the surface of the skin - flaccid, with thin covers, flat. Then the tire quickly breaks, revealing erosions. Also, the tire can dry out with the skin and form thin crusty scales that look like plates. They grow on each other and this phenomenon is a short time spreads throughout the skin. Pemphigus continues as erythroderma with large-plate peeling. In this form of the disease, the mucous membranes are generally not affected. This illness can last for several years.

The first symptoms seborrheic pemphigus lesions appear on which there are yellow scales or brown crusts. They are fairly easy to remove and have small white spikes on the surface below. Such lesions appear on the face, sometimes on the hairy part of the head. If the crusts are removed, wet erosions remain on the surface. Later, blisters begin to appear on the back and chest, and less often on the arms and legs. The bubbles pass quickly, leaving brown crusts in their place. In this case, the patient may not even notice the appearance of blisters: the formation of crusts occurs very quickly. Symptoms are similar to the flow process seborrheic eczema. The disease can last a very long time, its course is usually benign.

Diagnosis of pemphigus

To make a diagnosis of pemphigus, it is necessary to study clinical picture. The specialist is also guided by the presence of positive Nikolsky's symptom, which determines acantholysis. This phenomenon suggests that while pulling on the remains of the bladder tire, the erosion becomes more voluminous due to the process perifocal detachment of epidermal cells. The diagnosis also confirms the presence of acantholytic cells, which, due to acantholysis lost touch with each other. These cells are found in fingerprint smears taken from the surface of fresh erosions. Acantholytic cell has a large nucleus that occupies almost the entire cell; it is also characterized by heterogeneous staining cytoplasm.

If the disease is severe, then a positive Nikolsky symptom is detected even during the examination of skin without lesions. To do this, simply rub the skin with your fingers. Near the blisters and even far from them, the upper layers of the skin peel off and erosion appears. When pressing on the bubble, it also happens perifocal epithelial detachment.

If the diagnosis is difficult to establish, then in a dermatological hospital or in a skin and venereal disease clinic, histological examination skin with lesions or mucous membrane. For this purpose, a special immunofluorescence reaction is used to detect antibodies, which belong to class G immunoglobulins. When diagnosing pemphigus, it is important to differentiate it from Gougereau-Hailey disease, that is, benign seminal pemphigus. With this diagnosis, acantholysis is weakly expressed, and Nikolsky's symptom is negative.

Treatment of pemphigus

Treatment of all forms of this disease in mandatory carried out in a dermatological hospital. The treatment package includes taking corticosteroid drugs, which should be taken in large doses. Patients are also prescribed cytostatics and other drugs. Such patients must be constantly registered with a specialist dermatologist, and regularly, despite the cure, take corticosteroid drugs. For this purpose, a special maintenance dose of funds is prescribed. Treatment with corticosteroids is usually combined with anabolic drugs. steroid hormones, as well as drugs calcium, potassium, deoxyribonuclease, ascorbic acid. It is unacceptable to cancel corticosteroid therapy under any circumstances, otherwise this can lead to an exacerbation of pemphigus. Thanks to the application modern methods Treatment of this disease was able to reduce mortality three times.

Viral pemphigus

Viral pemphigus is a very common childhood disease. It manifests itself as swelling in the mouth, feet, and hands. In some cases, tumors also appear on the buttocks and legs. If swelling appears in the mouth, it may make it difficult to eat. However, the disease is not dangerous and goes away on its own after a week. Typically, viral pemphigus most often appears in the fall and spring. Causes the appearance of viral pemphigus in children enterovirus(i.e. intestinal virus). It can be spread very easily by coughing and sneezing. The virus can also be contracted through the stool of a sick child. Symptoms of the disease usually appear several days after exposure to the virus.

An infected child may feel very tired. He may have a sore throat and a rise in body temperature. After about a day, wounds appear in the above mentioned places. Sometimes a rash appears before the blisters appear. Special treatment not required - the disease goes away on its own. During illness, it is important to avoid hot, sour and spicy foods to prevent irritation of the mouth.

Prevention of pemphigus

To prevent pemphigus, maintenance therapy with corticosteroids is used, which must be used for a long period. It is important to constantly monitor blood and urine sugar levels, arterial pressure . To prevent the spread of pemphigus, you should wash your hands as often as possible and adhere to other personal hygiene rules.

True pemphigus– a chronic disease of an autoimmune nature, which is characterized by the appearance of blisters on clinically healthy skin and mucous membranes. Features clinical course pemphigus allowed clinicians to isolate following forms diseases: vulgar, erythematous, vegetative and leafy. Pemphigus is diagnosed by detecting acantholytic cells in the impression smear and histological identification of blisters located inside the epidermis. In the treatment of pemphigus, the main thing is a course of glucocorticosteroids; it is successfully combined with methods of extracorporeal hemocorrection: plasmaphoresis, hemosorption, cryoapheresis.

General information

True pemphigus– a chronic disease of an autoimmune nature, which is characterized by the appearance of blisters on clinically healthy skin and mucous membranes.

Causes of pemphigus

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

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

Clinical manifestations of pemphigus

Pemphigus has a long, undulating course, and the lack of adequate treatment leads to disruption of the patient’s general condition. At vulgar form pemphigus blisters are localized throughout the body, have different sizes and are filled with serous contents, while the covering on the blisters is sluggish and thin.

Pemphigus foliaceus clinically manifested by erythematous-squamous rashes, thin-walled blisters tend to appear on previously affected areas; after opening the blisters, a bright red eroded surface is exposed, upon drying of which lamellar crusts are formed. Since with this form of pemphigus blisters also appear on the crusts, the affected skin is sometimes covered with a massive layered crust due to the constant separation of exudate. Pemphigus leaf affects the skin, but in very rare cases, lesions of the mucous membranes are observed; it quickly spreads throughout healthy skin and at the same time there are blisters, crusts and erosions on the skin, which merge with each other to form an extensive wound surface. Nikolsky's symptom is positive even on healthy skin; with the addition of pathogenic microflora, sepsis develops, which usually causes the death of the patient.

Pemphigus vegetans proceeds more benignly, sometimes patients long years are in satisfactory condition. The blisters are localized around natural openings and in the area of skin folds. Opening up, the bubbles reveal erosions, at the bottom of which soft vegetations with a fetid odor are formed; the vegetations are covered on top with a serous or serous-purulent coating. There are pustules along the periphery of the formations, and therefore vegetative pemphigus must be differentiated from vegetative chronic pyoderma. Nikolsky syndrome is positive only near the affected skin, but in the terminal stages pemphigus vegetans is similar to pemphigus vulgaris in its clinical manifestations.

Diagnosis of all types of pemphigus

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

Principles of pemphigus treatment

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

All patients with pemphigus should be monitored by a dermatologist; a gentle regimen, lack of physical activity and avoidance of sun exposure are recommended. Frequent changes of underwear and bed linen prevent secondary infections.

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

The prognosis of pemphigus is always unfavorable, since in the absence of adequate treatment, the death of patients occurs quite quickly from additional complications. Long-term hormone therapy in high doses increases the risk of side effects, but when you stop taking glucocorticosteroids, pemphigus begins to recur.

Pemphigus of newborns

Pemphigus of newborns is an acute, highly contagious infectious skin disease, clinically manifested in the form of pustules that spread very quickly over the skin. Unlike true pemphigus, neonatal pemphigus is bacterial in nature and its causative agent is Staphylococcus aureus.

In the pathogenesis of pemphigus in newborns, an important place is occupied by the reactivity of the skin of newly born children, which intensifies with birth injuries, prematurity and the wrong lifestyle of the pregnant woman. In response to the action of bacterial factors, blisters form on the skin and pemphigus of newborns is diagnosed. The epidemiology of pemphigus in newborns is a disorder hygiene standards in maternity hospitals, presence of outbreaks among staff chronic infection, autoinfection with pemphigus is possible if a newborn develops purulent diseases of the navel.

Pemphigus of newborns occurs in the first days of a child’s life, but the disease can occur 1-2 weeks later. On clinically healthy or slightly erythematous skin, small, tense, thin-walled blisters with serous contents appear. After a few hours, the process generalizes, the bubbles increase in size and open. In place of the blisters, painful erosions remain with remnants of the epidermis at the edges; the erosions are covered with serous-purulent crusts. With pemphigus of newborns, children experience symptoms of intoxication, fever, and many of them refuse to eat.

In the absence of adequate treatment, pemphigus in newborns provokes inflammatory processes in internal organs: pneumonia, otitis, phlegmon. In weak and very premature children, a septic form of pemphigus is possible, the mortality rate of which is quite high.

Newborn pemphigus is diagnosed based on visual examination; it must be differentiated from syphilitic pemphigus, which is a manifestation of congenital syphilis, in which the blisters are localized on the palms.

Antibiotic therapy has made it possible to significantly reduce the mortality rate from pemphigus in newborns, whereas previously more than half of sick children died; the prognosis for pemphigus in newborns is favorable with timely and adequate therapy. Aniline dyes and non-aggressive antiseptics are used locally.

Preventive measures include changing bedding and underwear, removing personnel with pustular rashes from work, monitoring pregnant women and timely local therapy for mothers with pustular rashes.

Pemphigus (syn.: pemphigus) is a group of bullous dermatoses in which the pathogenetic role belongs to circulating autoantibodies directed against antigens of the multilayer desmosomal apparatus system squamous epithelium(skin, mucous membranes of the oral cavity, esophagus and other organs).

Etiology and epidemiology of pemphigus

The development of pemphigus is observed in genetically predisposed individuals. The most significant is the association with certain alleles of the major histocompatibility complex (HLA) genes. IN different countries a correlation is found with various alleles of genes encoding HLA.
The disease develops under the influence various factors(reception medicines containing thiol groups; insolation; infectious agents; stress; use of certain food products; physical factors etc.), however, it is often not possible to determine the provoking factor. During the disease process, antigen-presenting cells recognize their own molecules that make up desmosomes, cancel T- and B-cell tolerance to their own autoantigens, and synthesize autoantibodies.

Autoimmune processes lead to the destruction of the connection between epidermal cells (acantholysis) due to the formation of IgG autoantibodies to the so-called “pemphigus” antigens (the most important of which are desmoglein 1 and 3) and their binding to glycoproteins cell membranes. Formed immune complexes subsequently cause destruction of desmosomes and the appearance of intraepidermal blisters.

The incidence of pemphigus in European countries and North America averages 0.1 to 0.2 cases per 100,000 population per year.

IN Russian Federation, according to Federal statistical surveillance, in 2014, the incidence of pemphigus was 1.9 cases per 100,000 adults (aged 18 years and older), and the prevalence was 4.8 cases per 100,000 adults.

Pemphigus classification

L10.0 Pemphigus vulgare
L10.1 Pemphigus vegetans
L10.2 Pemphigus foliaceus
L10.3 Brazilian bladderwort
L10.4 Pemphigus erythematous
Senir-Usher syndrome
L10.5 Drug-induced pemphigus
L10.8 Other types of pemphigus
Subcorneal pustulosis
Intraepidermal neutrophilic dermatosis

Symptoms (clinical picture) of pemphigus

All clinical forms of pemphigus are characterized by a long, chronic, wave-like course, which, in the absence of treatment, leads to disruption of the general condition of patients, and in some cases, to death.

Pemphigus vulgaris is the most common form a disease characterized by the presence of blisters of various sizes with a thin flaccid covering, with serous contents, appearing on apparently unchanged skin and/or mucous membranes of the mouth, nose, pharynx, and genitals.

The first rash most often appears on the mucous membranes of the mouth, nose, pharynx and/or red border of the lips. Patients experience pain when eating, talking, or swallowing saliva. A characteristic symptom is hypersalivation and a specific smell from the mouth.

After 3–12 months the process becomes more widespread with lesions skin. Bubbles persist for a short time (from several hours to a day). On the mucous membranes, their appearance sometimes goes unnoticed, since the thin covers of the blisters quickly open, forming long-term, non-healing painful erosions. Some blisters on the skin may shrink into crusts. Erosions in pemphigus vulgaris are usually bright pink in color with a shiny, moist surface. They tend to grow peripherally; generalization of the skin process is possible with the formation of extensive lesions, deterioration of the general condition, the addition of a secondary infection, the development of intoxication and fatal in the absence of therapy. One of the most characteristic features acantholytic pemphigus is Nikolsky's symptom, which is clinical manifestation acantholysis and is a detachment of the epidermis due to mechanical impact on the skin in the lesions, located next to them and, possibly, in distant areas of the skin.

Seborrheic or erythematous pemphigus (Senir-Usher syndrome), in contrast to pemphigus vulgaris, which often first affects the mucous membranes, begins on seborrheic areas of the skin (face, back, chest, scalp).

At the onset of the disease, erythematous lesions with clear boundaries appear on the skin, on the surface of which there are yellowish or brownish-brown crusts of varying thickness. The blisters are usually small in size and quickly dry into crusts, which, when peeled away, reveal a moist, eroded surface. The blisters have a very thin, flabby covering that lasts for a short time, so they often go unnoticed by patients and doctors. Nikolsky's symptom is positive mainly in the lesions. The disease may be limited in nature for many months and years. However, it is possible for the lesion to spread to new areas of the skin and mucous membranes (usually the oral cavity). When the pathological process generalizes, the disease acquires the symptoms of pemphigus vulgaris.

Pemphigus foliaceus is characterized by erythematous-squamous rashes, thin-walled blisters that reappear in the same places, when opened, pink-red erosions are exposed with the subsequent formation of lamellar crusts, sometimes quite massive due to the constant drying of the exudate that separates. Damage to the mucous membranes is uncharacteristic. Possible rapid spread of rashes in the form of flat blisters, erosions merging with each other, layered crusts, scales with the development of exfoliative erythroderma, deterioration of the general condition, and the addition of a secondary infection. Nikolsky's symptom is positive both in the lesions and on apparently healthy skin.

Pemphigus vegetans can proceed benignly for many years in the form of limited lesions if the patient’s condition is satisfactory. Bubbles most often appear on the mucous membranes of the oral cavity, around natural openings (mouth, nose, genitals) and in the area of skin folds (axillary, inguinal, behind-the-ear, under the mammary glands). At the bottom of the erosions, soft, juicy, foul-smelling vegetations are formed, covered with serous and/or purulent plaque with the presence of pustules along the periphery. Nikolsky's symptom is positive only near the lesions. In the terminal stage, the skin process resembles pemphigus vulgaris.

Pemphigus herpetiformis is a rare atypical bullous dermatosis, which in some cases clinically resembles Dühring's dermatitis herpetiformis. The rash may appear as plaques, with papules and vesicles surrounding the periphery, or as grouped papules, vesicles, or tense blisters, as in Dühring's dermatitis herpetiformis. Pemphigus herpetiformis is characterized by severe itching of the skin. In the absence of adequate therapy, the disease can progress and acquire signs of pemphigus vulgaris or pemphigus foliaceus.

Paraneoplastic pemphigus occurs against the background of neoplasia, and can also occur during or shortly after chemotherapy treatment for malignant neoplasms. In most cases, paraneoplastic pemphigus is combined with lymphoproliferative neoplasia, thymoma, sarcoma, carcinoma and solid cancers various localizations. As a rule, the clinical picture of paraneoplastic pemphigus is similar to that of pemphigus vulgaris with simultaneous damage to the skin and mucous membranes, but sometimes skin lesions atypical for the disease are observed, accompanied by itching and resembling exudative erythema multiforme, bullous pemphigoid or toxic epidermal necrolysis.

Drug-induced pemphigus (drug-induced) may resemble the clinical picture of pemphigus vulgaris, seborrheic or foliaceous pemphigus. Its development is most often associated with taking medications containing sulfhydryl radicals (D-penicillamine, pyritol, captopril) and antibacterial drugs of the β-lactam group (penicillin, ampicillin and cephalosporins) and is caused by biochemical rather than autoimmune reactions. In cases of drug-induced pemphigus, complete recovery is possible after discontinuation of the medication.

IgA-dependent pemphigus is rare group autoimmune intraepidermal bullous dermatoses, characterized by vesiculopustular rashes, neutrophilic infiltration, acantholysis and the presence of both fixed and circulating IgA autoantibodies directed to antigens of the intercellular binding substance of stratified squamous epithelium.

The clinical picture of IgA-dependent pemphigus, regardless of the type of its manifestation, is represented by flaccid vesicles or pustules located on both hyperemic and apparently “healthy” skin. Pustules tend to merge to form ring-shaped lesions with crusts in the central part. The rashes are most often localized on the skin in the area armpits, scrotum, torso, upper and lower limbs. Less commonly, the pathological process involves the skin of the scalp and behind the ear, as well as mucous membranes. Patients often complain of intense itching. As a rule, IgA-dependent pemphigus is more benign than IgG-dependent pemphigus.

Diagnosis of pemphigus

To make a diagnosis, the following studies are necessary:

clinical examination of the patient;
definition of Nikolsky's symptom;
cytological examination for acantholytic cells in impression smears from the bottom of fresh erosions of the mucous membranes and/or skin (the presence of acantholytic cells is not pathognomonic, but very important diagnostic sign; at the onset of the disease, especially with seborrheic pemphigus, acantholytic cells may be absent);
histological examination (allows you to detect the intraepidermal location of cracks and/or blisters);
indirect immunofluorescence method (allows you to detect circulating IgG autoantibodies against intercellular binding substance antigens), the patient’s blood serum is used for analysis;
direct immunofluorescence method (allows you to detect class G immunoglobulins in the intercellular adhesive substance of the epidermis in a biopsy of apparently healthy skin obtained near the lesion);
determination of antinuclear antibodies (for differential diagnosis of erythematous pemphigus).

To determine the patient's condition, identify possible complications previous therapy with glucocorticosteroids and other immunosuppressive drugs and prescription concomitant therapy The following studies are required:

clinical blood test (with mandatory determination of platelet levels);
biochemical blood test (determining the level of bilirubin, transaminases, glucose, creatinine, protein, potassium, sodium, calcium);
clinical urine analysis;
density determination bone tissue in accordance with clinical guidelines on diagnosis, prevention and treatment of osteoporosis;
chest x-ray;
ultrasonography internal organs.

In case of damage to the mucous membranes, consultations with an otorhinolaryngologist, ophthalmologist, gynecologist, or urologist are recommended (if there are appropriate indications). If side effects from treatment occur, consultations may be required with: a therapist, cardiologist, gastroenterologist, endocrinologist, psychiatrist, surgeon, traumatologist, phthisiatrician.

Differential diagnosis

Differential diagnosis is carried out with bullous pemphigoid, Dühring's dermatitis herpetiformis, chronic benign familial pemphigus Gougereau-Hailey-Hailey, cicatricial pemphigoid, discoid lupus erythematosus, seborrheic dermatitis, Lyell's syndrome, erythema multiforme, chronic pyoderma vegetans, etc.

Lever's bullous pemphigoid differs from pemphigus by the presence of tense blisters with a dense covering, rather rapidly epithelializing erosions (in the absence of secondary infection), the absence of Nikolsky's sign, the subepidermal location of blisters, the absence of acantholytic cells and the location of class G immunoglobulins along basement membrane epidermis.

Dühring's dermatitis herpetiformis is characterized by a polymorphic itchy rash, dense, tense grouped blisters on an edematous hyperemic base, rapid epithelization of erosions, the absence of Nikolsky's sign and acantholytic cells in the impression smear from the bottom of erosions, subepidermal location of blisters, deposition of immunoglobulins A in the area of the dermal papillae, high content eosinophils in cystic fluid and/or peripheral blood.

In chronic benign familial pemphigus Gougerot-Hailey-Hailey, the distinctive features are the familial nature of the lesion, a benign course, deterioration of the skin process in the summer, localization of lesions (lateral surface of the neck, axillary, inguinal folds, navel area), the presence of maceration of the skin with the formation tortuous fissures like “brain convolutions”, pathognomonic for this disease. Nikolsky's symptom is not always positive and only in lesions. Acantholytic cells are found, but without signs of degeneration, and immunoglobulin deposition is unusual. The disease occurs with periods of remission and exacerbation, mainly in the summer. Rashes often regress when prescribed only external therapy(without using medicines systemic action).

Cicatricial pemphigoid differs from pemphigus in the absence of acantholytic cells, negative symptom Nikolsky, the development of cicatricial changes on the oral mucosa, skin and conjunctiva, the subepidermal location of blisters, as well as the absence of IgG in the intercellular substance of the epidermis in an immunomorphological study.

Discoid lupus erythematosus is distinguished by a characteristic triad of symptoms in the form of erythema, hyperkeratosis and atrophy. Acantholytic cells and intraepidermal blisters are not detected. Nikolsky's symptom is negative.

With seborrheic dermatitis, there is no acantholysis, damage to the mucous membranes, or histological and immunofluorescent signs characteristic of pemphigus.

Lyell's syndrome (toxic epidermal necrolysis) is an acute disease accompanied by fever, polymorphism of rashes, extremely severe general condition and usually associated with taking medications. The disease is characterized by detachment of the epidermis with the formation of extensive painful erosions. Nikolsky's symptom is sharply positive. Possible damage to mucous membranes.

With multiform exudative erythema Along with spots and papules, vesicles, bubbles, and blisters may occur. Blisters form on the mucous membranes, which open to form painful erosions. An edematous ridge forms along the periphery of the spots and/or edematous papules, and the center of the element, gradually sinking, acquires a cyanotic tint (symptom of “target”, or “iris”, or “bull’s eye”). Subjectively, the rash is accompanied by itching. The rashes tend to merge to form garlands and arcs. The rash appears within 10–15 days and may be accompanied by a deterioration in the general condition: malaise, headache, fever. Then, over the course of 2–3 weeks, they gradually regress without leaving scars; in their place, pigmentation may be observed.

Chronic vegetative pyoderma, in addition to signs reminiscent of pemphigus vegetans, has symptoms of deep pyoderma: erosions, ulcers, deep folliculitis. Nikolsky's symptom is negative, and there are no paraclinical signs of pemphigus.

Sneddon-Wilkinson disease (subcorneal pustular dermatosis) is characterized by the development of superficial conflict pustules with a diameter of up to 1.0–1.5 cm with a flabby tire, located on a hyperemic background, a slightly edematous base, prone to grouping and herpetiform arrangement. Due to the fusion of elements, scalloped lesions are formed, along the periphery of which fresh elements appear, and in the central part of the lesion the rash is in the stage of resolution. The pathological process is localized mostly on the skin in the abdomen and limbs (flexor surfaces), in the armpits and under the mammary glands. From subjective sensations, in rare cases it is noted slight itching. The general condition of patients is usually satisfactory. The disease occurs in paroxysms with incomplete remissions. Circulating and fixed IgA in intercellular spaces stratified epithelium are not detected.

In some cases, it is necessary to carry out differential diagnosis between different forms of pemphigus.

Treatment of pemphigus

Treatment Goals

stabilization of the pathological process;
suppression of the inflammatory response;
epithelization of erosions;
achieving and maintaining remission;
improving the quality of life of patients.

General notes on therapy

The basis of treatment for pemphigus is systemic glucocorticosteroid drugs, which are prescribed to every patient with a confirmed diagnosis. They are prescribed according to vital indications, therefore absolute contraindications no for their use.

In addition to glucocorticosteroid drugs, it is possible to prescribe adjuvant therapy (azathioprine, etc.), which allows reducing their dose or overcoming resistance to glucocorticosteroid drugs. If indicated, symptomatic therapy is prescribed.

Indications for hospitalization

the appearance of fresh rashes on the skin or mucous membranes (ineffectiveness of therapy on an outpatient basis);
the presence of secondary infection in the lesions.

Effective methods for treating pemphigus:

Systemic therapy

Glucocorticosteroid drugs

Prednisolone is most often used; other glucocorticosteroid drugs (GCS) are prescribed in accordance with the prednisolone equivalent.

At the first stage, therapy is carried out with maximum doses of systemic corticosteroids (80–100 mg per day, but not less than 1 mg per kg of the patient’s body weight) for 3 weeks, less often – 4 or more weeks. At in serious condition The patient is also prescribed higher doses of GCS – up to 200 mg per day and higher. The daily dose of the drug is taken strictly in the morning (before 11.00). In this case, high doses of CGS can be taken in two stages at 7.00–8.00 and 10.00–11.00, but always after each meal. Oral administration high doses of GCS can partially replace it parenteral administration or by introducing prolonged forms of the drug (no more than once every 7–10 days).

Criteria for assessing the positive effect of treatment with systemic glucocorticosteroids for autoimmune pemphigus: absence of fresh bullous rashes; epithelization of 2/3 of the lesions with continued active epithelization of remaining erosive defects, in particular those located on the oral mucosa; negative Nikolsky phenomenon; absence of acantholytic cells.

The second stage of therapy is divided into 7 stages of dose reduction from 65 mg to 20 mg per day and corresponds to the average doses of systemic glucocorticosteroid drugs for pemphigus. The duration of the stage is 9 weeks.

The initial dose reduction of GCS is possible by 1/4–1/3 of maximum dose after achieving a clear therapeutic effect (cessation of the appearance of new blisters, active epithelization of erosions). It is not recommended to reduce the dose in the presence of active insolation, acute infectious diseases and exacerbations of chronic diseases.

At the third stage, the dose of systemic corticosteroids is reduced, starting from 20 mg per day.

Table 2.

Scheme for reducing the dose of systemic glucocorticosteroid drugs (prednisolone), starting with 4 tablets by day of the week.

	Mon	W	Wed	Thu	Fri	Sat	Sun
Week 1	4	4	4	4	4	4	4
Week 2	3,75	4	4	4	4	4	4
Week 3	3,75	4	4	3,75	4	4	4
Week 4	3,75	4	4	3,75	4	3,75	4
Week 5	3,75	4	3,75	3,75	4	3,75	4
Week 6	3,75	4	3,75	3,75	4	3,75	3,75
Week 7	3,75	4	3,75	3,75	3,75	3,75	3,75
Week 8	3,75	3,75	3,75	3,75	3,75	3,75	3,75

Thus, ¼ tablet of prednisolone is discontinued over 8 weeks, and a maintenance dose of 6.25–3.75 mg per day is achieved over 128 weeks. This scheme allows you to avoid relapse of the disease during the period of reducing the dose of the drug. The maximum permissible minimum maintenance dose can vary from 2.5 to 30 mg per day. Sometimes, in severe cases of pemphigus, the maintenance dose cannot be reduced below 40–50 mg per day.

The duration of treatment is determined individually; as a rule, therapy is carried out for life, and only in rare cases can the use of GCS be abandoned.

Cytostatic drugs

Used to increase the effectiveness of GCS therapy and reduce their course dose.

methotrexate 20 mg (if well tolerated, up to 25–30 mg)

During treatment, it is necessary to monitor clinical and biochemical blood tests and clinical analysis urine at least 1-2 times a week.

Immunosuppressants

Used when there is insufficient therapeutic effectiveness GCS and the presence of contraindications to the use of cytostatics:

cyclosporine 5 mg per kg body weight per day. Treatment with cyclosporine should be carried out strictly under the control of blood and urine creatinine
Azathioprine is prescribed in a daily dose of 100–200 mg orally. It is easier to tolerate by patients and rarely causes complications. Treatment with azathioprine should be carried out strictly under the supervision of a clinical blood test.

External therapy

The area of blisters and erosions is treated with a solution of aniline dyes, in the presence of a secondary infection - with aerosols containing corticosteroids and antibacterial drugs. Antiseptic rinse solutions are applied to erosions in the area of the oral mucosa.

Anabolic hormones, potassium and calcium preparations, vitamins (C, rutin, B2, pantothenic and folic acid). When secondary infections develop, antibacterial drugs are used.

Diet for pemphigus:

Frequent and small meals are recommended. When the oral cavity is affected, the patient’s diet includes pureed soups, as well as mucous porridges in order to prevent a complete refusal of food. The diet should include restrictions table salt, carbohydrates and at the same time contain proteins and vitamins.

Requirements for treatment results

stopping the appearance of new rashes;
epithelization of erosions;
elimination of infection in the affected areas.

Prevention of exacerbations of pemphigus

Tactics of outpatient patient management and preventive measures to prevent exacerbation of autoimmune pemphigus.

When improving general well-being, cessation of the appearance of fresh bullous rashes, complete or almost complete (2/3) epithelization of erosive defects, patients with pemphigus are transferred to outpatient treatment. After discharge from the hospital, such patients should be constantly under the supervision of a dermatovenerologist and related specialists.

The dose of systemic glucocorticosteroid drugs is subsequently reduced very slowly to a maintenance dose according to the schemes presented above, strictly under the supervision of a dermatovenerologist. During summer and periods of epidemics viral infections it is not advisable to reduce the dose of a systemic glucocorticosteroid drug. Before performing surgical interventions or dental prosthetics, it is necessary to increase the dose of the systemic glucocorticosteroid drug by 1/3 of daily dose for 3–5 days or conduct a course of betamethasone injections at a dose of 2 ml IM 5 days before the planned surgical intervention, before surgery and a week after it. Corrective therapy is mandatory.

Clinical examination and monitoring of laboratory parameters ( general analysis urine, clinical and biochemical tests blood, coagulogram) should be carried out at least 2 times a year, if necessary more often. The level of electrolytes in the blood, chest X-ray, densitometry, and ultrasound are monitored annually. abdominal cavity, kidney, thyroid gland, pelvic organs and mammography (in women), prostate (in men), esophagogastroduodenoscopy, tests for tumor markers, as well as consultations with related specialists (therapist, neurologist, endocrinologist, otorhinolaryngologist, dentist, etc.).

Patients may be allowed to work that does not require excessive physical and mental stress, and is not associated with hypothermia and exposure to the sun. Insolation is contraindicated for patients with all forms of pemphigus; it is necessary to constantly use sunscreens with the maximum degree of protection. It is important for patients to maintain a routine of work, rest and sleep. Changes in climatic conditions and treatment are not allowed mineral waters and mud at resorts, therapeutic and cosmetic massages.

Tactics in the absence of treatment effect

If there is no effect from the therapy, intravenous immunoglobulins, plasmapheresis, extracorporeal photochemotherapy can be used, and epidermal nerve growth factor can be used to accelerate the healing of erosions...

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I had such a bump on my lip, just as shown in the photo, only on the other side. As I noticed (after maybe 2-3 weeks), I consulted a doctor. Prescribed Hexal. After a long course, about 40 days, it passed. I hope this won’t happen again, it’s a very nasty infection.

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