Clinical case of pemphigus vulgaris in old age

What is pemphigus? Symptoms, causes and treatment

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

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

Causes

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

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

Mechanism of bubble formation

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

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

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

Routes of transmission

This type of viral disease can be contracted in almost any public place.

The most common methods of infection with viral pemphigus are:

• Airborne droplets - most often when visiting public places such as kindergartens and schools;
• Through contact with household items that have previously been touched by an infected person.

Thus, it should be noted that short-term contact of a healthy child with an infected person can cause infection with viral pemphigus.

Children especially often become infected with viral pemphigus in the absence of necessary food processing and failure to wash their hands after visiting the street.

Classification

Types of non-acantholytic pemphigus:

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

Classification of true pemphigus:

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

Pemphigus in newborns

The manifestation of viral pemphigus in newborns occurs as a result of reduced immunity and damage to the body by the staphylococcal virus.

Viral pemphigus appears in the form of red spots on the skin, sometimes blisters with clear liquid can be observed.

Viral pemphigus most often forms after the 20th day of a newborn’s life, since in the first days maternal immunity is still active, which reduces the risk of infection with pemphigus.

Causes of pemphigus in newborns:

• Weak immunity;
• Insufficient maternal hygiene;
• Infection during childbirth;
• Premature birth;
• Damage to the baby's skin.

Viral pemphigus disease is manifested by the following symptoms:

• Skin rashes;
• Peeling of the skin;
• Swelling;
• Inflammation of the umbilical cord;
• Wounds in places where a blister with liquid is damaged;
• Lack of appetite;
• Lethargy;
• Increased body temperature.

There are several stages of development of viral pemphigus in newborns:

• Erythematous stage - red spots appear on the skin with the release of colorless liquid;
• Exfoliative stage - manifested by the appearance of blisters in the arms, thighs and abdomen. It tends to pass quickly and does not require long-term treatment;
• Regenerative stage - blisters form on the newborn’s skin, which quickly become damaged and form small erosions.

Staphylococcal pemphigus is also isolated, which is formed as a result of the staphylococcus virus, which affects the skin.

Viral pemphigus in newborns is transmitted by airborne droplets and appears in children only through contact with an infected person.

Symptoms

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

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

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

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

Hereditary pemphigus (epidermolysis bullosa)

The paper outlines the clinical features of the most common types of pemphigus neonatorum (epidermolysis bullosa), diagnostic problems, the basic principles of treatment, potentialities of prevention. Particular emphasis is laid on a diet, care, treatment of complications, and topical therapy. IN AND. Albanova, Pharmaceutical Research and Production Enterprise "Retinoids", Chief Physician of the Diagnostic and Treatment Centre, Doctor of Medical Sciences. Sciences, dermatovenerologist. VI Albanova, MD, dermatovenereologist, Head Physician, RETINOIDS Therapeutical-and-Diagnostic Center, RETINOIDS Pharmaceutical Research and Production Enterprise. N

hereditary epidermolysis bullosa is a group of blistering hereditary skin diseases that includes more than 20 monogenic dermatoses. Since the question of the independence of many of them has not yet been resolved, it is customary to call them forms of hereditary epidermolysis bullosa. Clinically common to all forms is the early onset of the disease, most often from birth or the first days of life, and the occurrence of blisters or erosions on the skin and mucous membranes as a result of minor mechanical trauma (“mechanobullous disease”). The presence or absence of scars after healing provides the basis for dividing all forms into dystrophic and simple. With the introduction of electron microscopy into the diagnosis of hereditary epidermolysis bullosa, all forms began to be divided into 3 groups: simple, borderline and dystrophic epidermolysis bullosa. In simple forms of epidermolysis bullosa, the formation of blisters occurs as a result of cytolysis of basal epithelial cells, which is revealed on electron diffraction patterns in the form of pronounced swelling of their cytoplasm with rupture of the cell membrane. In this case, the intact basement membrane is located at the base of the bladder. In borderline forms, separation of the epidermis from the dermis occurs at the level of the light plate of the basement membrane of the epidermis due to the inferiority of hemidesmosomes and attachment filaments. A dense lamina of basement membrane is located at the base of the blisters. In dystrophic forms, the separation of the epidermis from the dermis occurs below the basement membrane and is associated with the inferiority of the attachment fibrils - the structures connecting the basement membrane with the dermis. To carry out treatment, it is enough to know the main clinical forms. However, issues of prognosis and genetic counseling can only be resolved on the basis of an accurate diagnosis, which is currently only possible in large diagnostic centers with specialists in hereditary skin diseases and electron microscopy of the skin (Central Dermatovenerological Institute of the Ministry of Health of the Russian Federation), as well as using the method immunofluorescence or immunohistochemical mapping. Let us briefly look at the main clinical signs of 6 significantly different nosological forms of hereditary pemphigus.

Simple generalized epidermolysis bullosa

The disease is inherited in an autosomal dominant manner and manifests itself from birth or the first month of life. The first blisters appear on the feet, less often on the hands, subsequent ones - in places of pressure from clothing and shoes, as well as friction (neck, lower back, elbows, knees). With age, the number of rashes decreases, which is apparently due to the ability of patients to avoid mechanical injuries. Exacerbation always occurs in the warm season, although in young children seasonal fluctuations are not always noticeable. Bubbles and erosions appear a short time after the injury (20 - 30 minutes). The blisters have a hemispherical shape, a dense elastic covering, serous or, less often, bloody contents (Fig. 1). A ring of hyperemia appears around the bubble. Subjectively, the appearance of a bubble is accompanied by a burning sensation and pain, especially intense in the first hours after its formation. Emptying the blisters brings relief to the patient and prevents further increase in their size. Healing occurs quickly (2 - 3 days), while the lining of the bladder dries out and peels off. If the covering of the bladder is cut off, the resulting erosion becomes covered with a crust and healing is somewhat delayed. The healing period is accompanied by itching. After healing, slight flaking and pigmentation may occur. The repeated appearance of bubbles in the same place is typical.

Rice. 1. Patient N., 19 years old. Simple generalized epidermolysis bullosa. Fresh hemispherical bubble with serous contents.

All patients have hyperhidrosis of the palms and soles. Gradually, focal hyperkeratosis forms at the sites of pressure and friction on the soles. Changes in the nail plates (yellowish-gray color, thickening, curvature) are observed in all adult patients. Mycoses of the feet are often associated with the underlying disease.

Rice. 2. Patient K., 8 years old. Epidermolysis bullosa herpetiformis simplex. Typical lesions are round in shape with pigmentation in the center and blisters and crusts along the periphery.

In young children, blisters sometimes appear on the oral mucosa, but due to the rapid healing of erosions, they often go unnoticed, and when infected, aphthous stomatitis is diagnosed.

Simple localized epidermolysis bullosa

The disease is characterized by the same symptoms as the previously described form, but the rashes are located exclusively on the hands and feet. The literature describes cases where the first signs of the disease occurred much later than usual - in adults during military service or agricultural work with increased load on the feet. In some patients, the disease begins with widespread rashes, which gradually turn into localized ones, which indicates the genetic commonality of generalized and localized epidermolysis bullosa simplex.

Epidermolysis bullosa herpetiformis simplex

It is inherited in an autosomal dominant manner, but sporadic cases are common. The disease begins at birth or the first week of life. The first rashes are usually located on the hands and feet, they spread quickly, which is accompanied by a disturbance in the child’s general condition (loss of appetite, anxiety, sleep disturbances, and often a rise in temperature). The blisters quickly erode, and areas devoid of epithelium sometimes occupy most of the skin. Secondary infection of erosions and dehydration develop. By 3-6 months of age, the rashes acquire a typical appearance for this form - hemispherical blisters with a tense tire and serous, hemorrhagic or purulent contents, as well as erosions covered with crusts, forming ring-shaped, arched, scalloped foci, in the center of which there is a pigmentation zone ( Fig. 2). Each individual erosion heals quickly, the healing of the entire lesion lasts for 1 - 1.5 months.

Rice. 3. Patient S., 10 years old. Dominant dystrophic epidermolysis bullosa. Blisters and erosions in frequently injured areas, skin atrophy on the legs and knees.

Most often, lesions are located around the mouth and nose, on the hands, feet, and knees, but any area of ​​the skin can be affected.

The progression of the pathological process can be observed from several months to a year, after which a gradual improvement occurs - a decrease in the area of ​​skin lesions, a less frequent occurrence of blisters and infected rashes, and normalization of the general condition. From 2 to 3 years of age, patients experience a distinct seasonality of exacerbations - in the summer there are more rashes. Sometimes complete resolution of the rash is observed when the body temperature rises above 38° C during infectious diseases. By the end of the first ten years of life, the occurrence of blisters becomes rare, and in the second it stops.

Rice. 6. Patient Zh., 7 years old. Recessive dystrophic epidermolysis bullosa. Contractures and syndactyly of the hands.

All patients have hyperkeratosis of the feet, which gradually increases with age. After the blistering stops, it sometimes becomes the only symptom of the disease. The oral mucosa is affected in most patients. Epithelization of erosions in the mouth occurs very quickly, leaving no traces. Caries, less often defects of tooth enamel and anomalies in the position of teeth, are present in all patients over 2 years of age. A variety of nail changes are observed in all patients and progress with age. In the first year of life, blisters sometimes appear under the nail plates, which peel off, but always recover. Children's growth and development are appropriate for their age.

Severe borderline epidermolysis bullosa

The disease is the most severe form of hereditary pemphigus, leads to the death of the child in the first weeks or months of life, and is inherited in an autosomal recessive manner. Rashes in the form of flaccid, easily eroded blisters immediately become generalized. Characteristic features are the presence of granulations along the edges of erosions, especially pronounced on the face and nail folds, severe general condition of the child, and severe anemia. Often, epidermal detachment occurs without the formation of a bubble. Healing is slow, with skin atrophy. There are always erosions in the oral cavity. Bubbles can also form in the esophagus, larynx, pylorus, small and rectal intestines, gall bladder, urethra, and kidneys. Patients are sharply retarded in physical development. Death occurs as a result of asphyxia from a detached bladder or dermatogenous sepsis.

Dominant dystrophic epidermolysis bullosa

The disease is inherited in an autosomal dominant manner and begins at birth or the first days of life. In the first months, skin damage is generalized, later blisters usually appear on the same frequently injured areas: hands, feet, knees, elbows, neck. Healing occurs with the formation of an atrophic scar with a clear boundary, slight folding of the skin in the scar area, and pigmentation (Fig. 3). The nail plates are affected in all patients, and only in rare cases are the nails absent; more often they are dystrophic. The growth and development of children is not impaired. At an early age, obstruction of the esophagus may occur, which is expressed in choking, vomiting when eating solid food, drooling, and pain when swallowing. These phenomena are reversible. With age, blisters appear less and less often, and in adults the presence of the disease can only be reminded of dystrophic changes in the nails and barely noticeable scars on the elbows, knees and ankles.

Recessive dystrophic epidermolysis bullosa

The disease is inherited in an autosomal recessive manner, has a severe course, and often leads to death at an early age. The disease always occurs from birth or the first hours of life. Already at birth, the skin of the extremities is often eroded. In the first days of life, the rash spreads, and blisters appear not only as a result of minor skin trauma, pressure and friction, but also spontaneously (Fig. 4). Even large erosions heal relatively quickly (depending on the size in 3 - 10 days), but new ones constantly appear (Fig. 5). Healing occurs with the formation of atrophic scars; contractures and syndactyly gradually develop on the hands and feet (Fig. 6,7). Nail plates are absent from birth or are gradually lost as a result of the formation of subungual blisters. Cicatricial atrophy of the skin of the scalp is manifested by diffuse sparseness of hair and its dystrophic changes. Multiple blisters also appear on the mucous membrane of the oral cavity, esophagus, and rectum. The process of scarring in the mouth leads to limited mobility of the tongue, atrophy of its papillae, fusion of the vestibular folds and microstomia, in the esophagus - to its narrowing, impaired passage of food, in the rectum - to chronic constipation, sharp pain during defecation. The teeth are affected in all patients, caries, tooth enamel defects, and location anomalies predominate. Damage to the mucous membrane of the eyes is often observed in childhood, which is clinically manifested by burning and pain when trying to open the eyes. Epithelization of conjunctival erosions occurs quickly (2 - 4 days). Scarring on the cornea ends with the formation of cloud-like opacities that do not significantly impair vision. The general condition of patients is characterized by weakness, fatigue, and long periods of low-grade fever. Constant painful sensations lead to limited mobility of patients, slower psychomotor and physical development, and social maladjustment. Hypochromic anemia is noted. With age, the ability to heal erosive and ulcerative lesions decreases; some lesions do not heal for several months or even years. On such lesions, as well as on scars, epithelial tumors can form, most often squamous cell carcinoma, resistant to therapy. Among the causes of death in the first year of life, the most common are asphyxia, aspiration pneumonia, dermatogenous sepsis, and at the age of over 30 years - malignant skin tumors.

Treatment of hereditary pemphigus

When developing treatment tactics, it should be taken into account that the period of activity of hereditary diseases stretches for many years and it is hardly worth recommending an equally long-term use of even effective drugs. Active therapy is used during exacerbation and the occurrence of complications in short courses, and then is replaced by a longer prescription of restorative and symptomatic drugs. Particular attention is paid to issues of care and nutrition. The main goals of treatment are to prevent the appearance of blisters on the skin and mucous membranes, accelerate the healing of existing rashes, prevent and treat secondary infections, prevent and treat severe complications associated with scarring.

Pathogenetic therapy

It is known that the skin of patients with recessive dystrophic epidermolysis bullosa produces an excessive amount of structurally altered collagenase. In this regard, the use of drugs that inhibit the production or activity of collagenase - diphenin (phenytoin), erythromycin, large doses of vitamin E and retinoids - is pathogenetically justified. Difenin

(used in psychiatry for the treatment of epilepsy) is produced in tablets of 0.1, administered orally twice a day at the rate of 3.5 mg/kg body weight per day in adults and 8 mg/kg in children.
During the first 3 days, 1/3 of the daily dose is prescribed, from the 4th to the 6th day 2/3, from the 7th day - the full dose. If it is necessary to discontinue the drug, reduce its dose in the reverse order. A positive effect in the form of a reduction in the number of blisters, accelerated epithelization of erosions, and increased skin resistance to traumatic influences is noted after 3 - 4 weeks. When treated with diphenin, dizziness, agitation, nausea, vomiting, tremor, lymphadenopathy, and gingival hyperplasia are possible. Erythromycin
is prescribed orally in the usual age-specific dose for 10 to 14 days.
Considering the bacteriostatic effect of the antibiotic, it is better to prescribe it when multiple blistering rashes on the skin are accompanied by secondary infection of erosions. Tocopherol acetate
(vitamin E) is available in capsules of 50 and 100 mg and in an oil solution of 5%, 10% and 30% (50, 100 and 300 mg/ml).
The drug for epidermolysis bullosa gives a positive effect only in doses exceeding 1500 mg per day. Children are prescribed 1/2 - 1/3 of this dose (that is, 500 - 1000 mg per day). The course of treatment is 20 - 40 days. The drug is prescribed in equal parts in the morning and evening during or immediately after meals. For older children who do not have swallowing problems and adults, vitamin E is prescribed in capsules, in other cases - in drops. We have not noted any side effects when using such high doses and have not described them in the literature. From the group of retinoids, tigazone and retinol palmitate
or retinol acetate are used. In addition to the anti-collagenase effect, they have the ability to accelerate epithelization. In this regard, it is better to prescribe them to patients with a significant area of ​​​​damage, but without secondary infection. Tigazone is available in capsules of 10 and 25 mg, prescribed in a daily dose of 1 mg/kg body weight (in equal parts 3 times a day with meals). If the effect is good (usually on the 3rd - 7th day), after 2 - 3 weeks the daily dose is reduced to 0.3 - 0.5 mg/kg. Long-term use of tigazon is not recommended, since the drug has many side effects: cheilitis, dry skin and mucous membranes, hair loss, biochemical disorders, and in children - growth retardation. Tigazon is embryotoxic and teratogenic and is contraindicated in pregnant women and women planning pregnancy. Before starting treatment, you must make sure that there are no abnormalities in clinical and biochemical blood tests. Retinol palmitate is produced in capsules of 100,000 IU and an oil solution of 100,000 IU/ml, retinol acetate is produced in capsules of 3300, 5000 and 33000 IU and an oil solution of 100,000 and 250,000 IU/ml. Both drugs are used in a daily dose of 5000 IU/kg body weight (equal parts in the morning and evening after meals). Compared to tigazon, the drugs are less toxic, but their positive effect appears at a later date (on the 7th - 10th day). The course of treatment is 1.5 - 2 months. Drugs with anticollagenase activity are not always effective. Most of them are not recommended for long-term use; usually 2-3 courses of treatment are carried out per year. The basis of treatment remains symptomatic therapy.

Symptomatic therapy

General therapy includes the use of broad-spectrum antibiotics for secondary infection of rashes (they are prescribed only when the general condition of the patient is disturbed), antihistamines and sedatives for severe itching, anabolic and restorative agents and enzymes for retarded physical development. In case of anemia, the best effect is obtained by direct blood transfusion, transfusion of red blood cells, plasma, albumin is possible, and an appropriate diet must be prescribed. In complex therapy, it is necessary to prescribe multivitamin preparations. Preference is given to complexes containing microelements. If the oral mucosa is affected, after each meal it is recommended to rinse your mouth with decoctions of chamomile, sage, calendula, St. John's wort, oak bark, and serpentine rhizome. After rinsing, it is good to use sea buckthorn oil, ointment with retinol palmitate, carotoline, solcoseryl, and Kalanchoe juice in the form of applications. Young children are given sea buckthorn oil in drops orally. For spasms of the esophagus with impaired passage of food, bed rest, dry heat on the chest area, antispasmodics, and electrophoresis with peloidin are recommended.

Skin care and external treatment

For constipation, you should not use laxatives that cause chemical or mechanical irritation of intestinal receptors; the best effect is achieved by diet and the introduction of warm vegetable or petroleum jelly into the rectum in the form of a microenema. The use of corticosteroid drugs, either internally or externally, is not recommended. Without preventing the formation of blisters or promoting healing, they enhance the formation of atrophic scars. Preventive vaccinations are contraindicated only during periods when the child’s general condition is impaired. From the first days of a sick child’s life, one should strive to minimize factors that injure the skin. Diapers should be soft, without seams. When swaddling, pay attention to ensure that the baby’s legs do not touch each other. All underwear is worn with the seams facing out. Clothes should not have elastic bands or ribbons that restrict movement. For the same reason, it is undesirable to use diapers. The child is picked up very carefully, only dressed or wrapped in a diaper, supported from below, avoiding stretching the skin. When a child begins to walk, it is advisable to hem his clothes (on his elbows and knees) with several layers of soft fabric. At home, it is recommended to wear loose clothing and no shoes. Walking shoes should be loose and easy to put on. All persons involved in caring for the child and monitoring his health should be warned about the characteristics of the disease and the inadmissibility of even minor trauma to the skin and mucous membranes (for example, during examination by a pediatrician, otolaryngologist or dentist). Blisters form more easily on dry, atrophic skin with reduced sebum secretion. In this regard, the skin needs to be artificially fattened and hydrated daily so that it becomes soft and elastic and is more resistant to mechanical stress. For this purpose, creams and ointments on a water-emulsion basis (ointment with retinol palmitate, ointment Radevit, 5% ointment with urea) or vegetable oil (except sunflower) are used. The cream used should not be thick. Blisters that form on the skin are emptied daily by pricking both sides with a thick injection needle and removing the fluid either by pressing or suctioning with a syringe. It should be remembered that your own epidermis is the best covering for the wound and most contributes to its healing. However, in most cases, repeated mechanical trauma leads to rupture of the exfoliated epidermis, exposing the erosive surface. It is best to immediately apply a collagen sponge covering (colaspon, algicol, digispon, etc.) to the erosions. If there are no coverings at hand, it is necessary to treat the wound with an antiseptic to avoid infection and apply a sterile bandage with wound-healing agents (ointments with retinol palmitate, Radevit, solcoseryl, Actovegin, Bepanten, panthenol aerosol).

Despite precautions, most erosions become infected and turn into ulcers.
(dermazin, levosin, levomekol, fastin)
are applied to infected areas Aerosol external agents are convenient:
olazol, hypozol, legrazol, levovinisol, etc.
Collagen sponge coatings containing enzymes are used to heal ulcers. In case of extensive damage, general UV irradiation at suberythemal doses has a good effect. Magnetic therapy is prescribed for selected long-term non-healing ulcers. Baths with herbs stimulate healing well, improve well-being and are the most pleasant procedure for patients of any age. For baths, any herbal decoctions with anti-inflammatory and astringent effects are used. For severe itching, add mint or valerian. After a bath, be sure to lubricate the skin with a nourishing cream. In many patients, milia form after healing, especially often on the back of the hands. These rashes resolve on their own over time and do not require treatment. Adult patients should be warned about the possible appearance of unusual rashes on scars and ulcers, which requires immediate contact with a dermatologist. Epithelial tumors in epidermolysis bullosa may be resistant to treatment; treatment should be started as early as possible.

Nutrition

At all periods of a patient’s life, nutrition must compensate for the loss of protein, salts and water associated with the formation of blisters. Breastfeeding is preferable; with the introduction of supplementary feeding and complementary feeding, the amount of protein introduced with food should be increased by 20%. Fruit juices and purees should not be sour. In the morning, it is useful to give the child a little unrefined vegetable oil, which compensates for the lack of polyunsaturated fatty acids and facilitates bowel movements. The diet includes foods rich in coarse plant fiber (cabbage, zucchini, beets, dried fruits). Food that injures the mucous membrane is prohibited (caramel, crackers, dryers, waffles, etc.). Adult patients are warned against drinking alcoholic beverages and spicy foods, which provoke the formation of blisters in the esophagus.

For spasms and narrowing of the esophagus, a mechanically and thermally gentle diet is used, increasing the number of meals.

Prof. S.I. Vozdvizhensky (Moscow Research Institute of Pediatrics and Pediatric Surgery) developed a method of surgical treatment of contractures and syndactyly of the hands in children with epidermolysis bullosa. However, there is a danger of relapse, and therefore a long period of anti-relapse therapy is necessary. Sick children can become disabled, adults are able to work or are disabled groups I - III, depending on the form, severity of the disease and complications. Due to the difficulties of treatment, prevention methods are of particular importance. Genetic counseling is possible after an accurate diagnosis has been established. The risk of developing the disease with dominant forms is 50% for each child. If healthy parents have a child with a recessive form of epidermolysis bullosa, then for each subsequent child the risk of developing the disease is 25%. If a patient with a recessive form wants to have a child, then his risk of developing the disease is negligible. The risk becomes greater (50%) if the second parent is a carrier of the identical recessive gene for epidermolysis bullosa. The risk increases significantly with consanguineous marriages. In case of borderline and recessive dystrophic epidermolysis bullosa, prenatal diagnosis is possible by fetal skin biopsy at 16–18 weeks of pregnancy, followed by electron microscopic examination. Such a study can be carried out at the Center for Maternal and Child Health (Moscow) and in a number of foreign countries. If the fetus has a disease, the pregnancy is terminated.

Literature:

1. Bruckner, Tuderman L. Epidermolysis bullosa hereditary. Hautarzt 1995;46:61-72. 2. Albanova V.I. Clinical characteristics of dominant dystrophic epidermolysis bullosa. Bulletin of dermatol. - 1994;1:48-52. 3. Albanova V.I. Epidermolysis bullosa. In the book “Monogenic dermatoses”. — Yoshkar-Ola. - 1993. P. 104-26. 4. Suvorova K.N., Albanova V.I. Hereditary epidermolysis bullosa. In the book “Children's dermatovenerology. - Kazan. 1996. pp. 69-80.

Diagnostics

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

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

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

How to determine pathology?

The diagnosis of pemphigus is made after interviewing the patient, as well as based on the totality of the results of the following examinations:

• histology;
• cytology;
• immunogram.

Pemphigus vulgaris, like other forms of vesicular dermatoses, is characterized by a positive Nikolsky symptom: when rubbing the affected mucous membranes or skin, the top layer of cells peels off due to acantholysis. Pemphigoid does not give such a reaction.

Diagnosis of viral and microbial forms involves determining inflammatory changes and antibodies to pathogens in the blood. It is recommended to take a blood test for sterility.

Treatment of viral pemphigus

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

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

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

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

Since children with this diagnosis are usually treated in a hospital setting, to enhance the therapeutic course, therapeutic procedures can be carried out aimed at clearing the blood of antibodies:

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

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

How to treat other forms of pemphigus?

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

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

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

Disease prevention

To prevent the development of viral pemphigus, it is recommended to follow simple prevention methods:

• Children at any age should have their own personal hygiene equipment. This is especially true for toothbrushes, washcloths and towels. The towel must be changed every two days. Before this, iron the item at high temperature;
• You should maintain proper nutrition and exclude harmful foods. The diet should contain all the necessary vitamins and minerals to improve immunity;
• The children's room must be kept clean;
• Clothing must be clean and changed daily;
• Avoid contact with infected people;
• Maintain proper hygiene, especially after visiting public places;
• Treat all emerging diseases in a timely manner, do not self-medicate.

Following these simple preventive measures can reduce the likelihood of contracting viral pemphigus. However, parents should be aware that no one is protected from the risk of becoming infected with pemphigus.

Medicines for the treatment of pemphigus

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

• Metipred;
• Prednisolone;
• Dexamethasone;
• Polcortolon.

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

• Metipred-depot;
• Diprospan;
• Depo-Medrol.

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

Possible complications during treatment:

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

If the patient’s condition sharply worsens while taking corticosteroids, the following measures may be recommended:

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

In parallel with glucocorticoids, cytostatics and immunosuppressants are prescribed to increase the effectiveness of therapy and the possibility of reducing doses of hormonal agents.

The following medications can be used for this:

• Sandimmune;
• Methotrexate;
• Azathioprine.

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

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

Complications

Pemphigus has an unpredictable course, often becoming chronic and resistant to therapy. Pemphigus vulgaris sometimes leads to the development of a secondary infection: encephalitis, meningitis, pneumonia, damage to the heart and joints. Dermatosis of newborns is dangerous due to the possibility of sepsis.

Local complications are non-healing suppurating erosions and ulcers, chronic infections of the oral cavity and upper respiratory tract.

The disease, which occurs in a severe form, requires the use of potent medications. Due to the need for long-term hormone therapy, the following problems arise:

• Itsenko-Cushing syndrome;
• diabetes;
• ulcerative lesions of the stomach and intestines;
• arterial hypertension;
• osteoporosis;
• mental disorders;
• exacerbation of chronic diseases.

Preventive measures

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

Important:

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

Measures to prevent pemphigus in newborns:

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

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

Recommendations for caring for a sick child

If a child develops symptoms of the disease, it is necessary to call a doctor. Once a diagnosis is made, a certain type of care must be provided to the patient.

This care is as follows:

• The child is bathed once every few days, adding special herbal decoctions to the water;
• Eliminate fatty, salty, sour foods from the diet;
• It is necessary to change household items and personal towels daily;
• Home clothes should not be too tight and have a synthetic composition;
• Bed linen must be changed every three days;
• Every day, the room where there is a person suffering from viral pemphigus must be cleaned and ventilated;
• Isolate the patient from other family members for at least 7 days;
• Treat skin lesions in a timely manner;
• Make sure that no bubbles are scratched.

Proper parental care can much more quickly reduce the unpleasant symptoms of viral pemphigus in children and also prevent the development of complications.

Forecast

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

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