Pemphigus in children: symptoms, diagnosis, treatment

Types of pemphigus

People distinguish between true pemphigus (pemphigus) and other blisters on the skin. The true one is of the following types:

• ordinary, or vulgar - occurs most often. Externally intact and undamaged skin becomes covered with blisters with serous fluid inside, which burst easily and quickly and heal. Localization - the mouth area and the mucous membrane of the lips, as well as the nasolabial triangle. At first glance, harmless bubbles appear more and more often, each time occupying a larger area throughout the body. When they burst, they leave pink eczema in this place. The child should begin receiving adequate therapy between 6 months and 2 years. Otherwise, there is a danger of death;
• vegetative - the course of the disease differs from the usual type only in the consequences on the skin. In this case, instead of eczema, vegetation papillomas of a grayish tint remain. These papillomas are growing;
• leaf-shaped - got its name due to the visual appearance of the crusts formed after the bursting of bubbles. A distinctive feature of this species is the rapid development of pathology. In this case, the count is not in months, but in days. In this case, the skin peels off in whole pieces shaped like leaves;
• erythematous - is a complication after leaf-shaped and has no striking differences from the previous form of pathology;
• seborrheic - originates in the hair on the head and face. Small bubbles quickly become a yellowish crust. The progression of the pathology is slow, affecting the back, abdomen and limbs. After peeling off the crust, weeping eczema remains.

There are other types of pemphigus that are not related to pemphigus:

• infectious viral pemphigus in children - develops under the influence of viruses: Coxsackie, enterovirus 71. In this case, the latter option may have the nature of an epidemic. It has specific localization sites - feet and palms, butt and genitals;
• congenital syphilis - occurs when there is intrauterine infection with syphilis. It appears within a couple of days after birth. The blisters burst quickly and leave behind weeping pink eczema.

Untrue types of pathology in medicine refer to the symptoms of pemphigus in children. This course of the disease allows for faster and more correct treatment of pemphigus in children, aimed at eliminating the root cause. The most difficult thing in this pathology is establishing the true cause of the disease.

A rare but severe type of true pemphigus is the paratumor type. It has the most striking symptoms. It occurs against the background of leukemia and lymphoma, and also appears as a harbinger of the development of a malignant tumor.

Spreading

Photo: Pemphigus vulgare
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The species' range includes the territory of Eurasia and America.

The insectivorous plant bladderwort lives in dark, swampy, tea-colored waters that are rich in tannins.

The ecological criterion for the common bladderwort species is lakes and reservoirs with a low nutrient content.

Botanical description

Utricularia vulgaris or popularly called "water bladder" is a medium-sized perennial aquatic bladder plant with bright yellow summer flowers and no root system. A drawing of Utricularia vulgaris is shown below.

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Pemphigus flower

Depending on the species, they live as epiphytes in a humid atmosphere, moist soil or aquatic environment. The leaves of the bladderwort are up to 16 centimeters in diameter, arranged in a rosette, thinly divided and interspersed with numerous blisters, which darken when eaten by insects. Flowers bloom above the surface of the water in mid-summer. During the transition from autumn to winter, it forms turions (winter buds), which settle to the bottom and help survive the winter.

The diameter of traps on leaves depends on the species and usually reaches 0.6-10 millimeters.

Features of the plant structure

It is a free-floating aquatic plant with “traps” on its leaves formed by membranous sacs or vesicles that are flat, small and pear-shaped. Each vial has a hole covered by a "hanging cap".

The gripping mechanism consists of four stiff bristles located close to the lower free edge of the “lid”. Then, when the insect comes into contact with these bristles, the hairs deform the bottom edge of the lid, causing it to open quickly. The water is “drawn” inside, taking the insect with it, and the lid closes behind it. Subsequently, a number of enzymes secreted by the inner wall of the vesicles and the population of bacteria located in them are digested by the animal.

Pemphigus blooms

Thus, the resulting organic and inorganic compounds are absorbed by the cell walls of the vesicles.

Aside from their strange traps, the common knotweed is also unique in that it has mostly undifferentiated tissue. That is, there is no clear distinction between the leaves, stem and root system.

Causes of occurrence and development

The pathology begins its development after the development of autoimmune antibodies aggressive to desmoglein proteins. These proteins act as an adhesive base used to connect skin cells. In the process of protein destruction, the skin is destroyed. At this point, bacteria from the environment begin to penetrate the skin layers, causing the formation of blisters.

The factors that trigger the pathological process still remain unclear. Some of them are established by science:

• genetic predisposition to autoimmune processes;
• malfunctions of the central nervous system;
• the presence of viruses, infections or other pathogens in the body - a connection has been identified between infection with endogenous bacteria and the appearance of an autoimmune reaction.

Russian official medicine identifies the following reasons causing an inadequate immune response:

• taking medications of the thiol group - strong antibiotics, immunomodulators and others;
• burns;
• herpes virus types 1, 2 and 8;
• tactile contact with pesticides;
• severe stress or nervous overload.

It is almost impossible to establish the true cause of the disease. In fact, in laboratory conditions only the presence of aggressive antibodies is determined.

Symptoms and signs

The early stages of the disease have virtually no dangerous signs. The child’s health remains normal, and skin changes have not yet appeared. The condition worsens as the area of ​​the affected skin gradually increases.

The bubbles themselves have a number of distinctive features - lethargy, easy to open, sagging skin under the liquid inside the bubble. Primary rashes are always localized in the mouth and mucous membranes of the lips. There are no painful sensations. Opened blisters form skin crusts or wet eczema, which take a long time to heal.

Gradually the affected area increases. Infection with bacteria leads to the appearance of suppuration and foci of inflammation, in place of which eczema and large erosions subsequently appear. Another distinctive feature is the lack of healing of the skin at the site of the blister rupture. Gradually, the erosions expand and unite into a single lesion.

All forms of pemphigus have a wave-like manifestation of symptoms. In the absence of a timely response, intoxication syndrome begins - nausea and headaches. Ultimately, the death of the little patient inevitably occurs. Viral pemphigus in children has symptoms similar to the true disease, and there is a pronounced viral infection.

When to see a doctor

Baby rash appears frequently and rarely poses a real threat to the child's life. Basically it becomes a reactive feature to allergens and infections. It is important for parents to know which rashes pose a mortal danger to the child. If signs of such a rash appear, you should seek help from a dermatologist.

The pediatric department of JSC "Medicine" (clinic of academician Roitberg) has been guarding children's health for many years. You can make an appointment with a dermatovenerologist by phone. The clinic has a convenient location, a two-minute walk from the Mayakovskaya metro station, as well as the possibility of placement in a hospital for examination and treatment.

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.

Prevention and prognosis

Medicine has not proposed any specific measures to prevent pemphigus due to the fact that the true causes of its occurrence have not been established. A set of preventive measures is aimed at strengthening the health of a little person:

• formation of strong immunity;
• hardening and walking in the fresh air from the moment of birth;
• proper nutrition;
• avoidance of excessive use of drugs and medications.

Forecasts depend on the speed of contacting a doctor - for symptoms of pemphigus in children, treatment should begin as early as possible. Therefore, you should not self-medicate under any circumstances. In order not to miss the appearance of signs of pathology, it is necessary to examine any skin problems with a dermatologist.

The prognosis of true pemphigus is a priori conditionally unfavorable. Even with adequate and timely treatment, the likelihood of death cannot be eliminated. Any form of pathology is considered chronic.

At the same time, the introduction of hormonal drugs into therapy made it possible to reduce the mortality rate from 65% to 7%. The disease requires constant maintenance therapy. Otherwise, 91% of cases experience acute exacerbations and sudden progression, and consequently an increase in the likelihood of death.

2. Reasons

As stated above, the etiopathogenesis of acantholytic pemphigus has not yet been precisely established.

Various hypotheses have been put forward and considered in this regard (viral, metabolic, neurogenic, toxic, hormonal, etc.), but convincing evidence has been obtained only that autoimmune mechanisms play a leading role in the development of pemphigus. However, the root causes of such an attack from one's own immunity, trigger factors and risk factors (with the exception of being female or belonging to a certain genetic line) are still unknown.

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Diagnostics

This pathology is rare, and pediatricians, as a rule, cannot determine it accurately. Therefore, the primary stage of diagnosis is the distinction between pemphigus and other dermatological diseases that are accompanied by rashes of blisters and blisters.

Differential diagnosis is carried out using a special method - Nikolsky's test. The essence of the test is to rub the skin near the bubble and on a distant area of ​​the skin, as well as pressing the fingertip on the bubble. A positive result is considered to be the presence of signs of skin separation:

• spreading of serous fluid into adjacent layers of skin when pressed;
• slight peeling of the skin in the form of a ribbon when you gently pull the skin over the bubble - similar to a sunburn;
• friction on a healthy area shows the mixing of the upper layers of the epidermis.

One of the most informative diagnostic methods is a blood test for the presence of aggressive antibodies to the desmoglein protein. If these antibodies are present, a diagnosis of pemphigus is made.

Additionally, the following may be assigned:

• cytological studies of serous fluid samples from the bottom of the erosion or from the bladder;
• general blood and urine analysis;
• chest x-ray;
• consultations with related doctors - nephrologist, cardiologist, infectious disease specialist.

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.

Treatment

How to treat pemphigus in a child? The main drugs today are hormones from the group of glucosteroids. In this case, systemic therapy is carried out without age restrictions.

Treatment of pemphigus has several main directions:

• preventing the appearance of new blisters and erosions;
• healing of affected areas of the skin.

Glucosteroids are administered in increased dosages. Due to this technique, the intensity of the formation of new foci decreases and the restoration process begins in existing erosions. This process takes about 2 weeks. Subsequently, the patient is transferred to maintenance hormonal therapy. The drug does not change, but the dosage is significantly reduced.

Viral pemphigus in children treatment is aimed at suppressing the virus. Pemphigus vulgaris requires the administration of a larger volume of drugs than foliaceus. The transition to maintenance therapy is carried out gradually. Moreover, the vast majority of patients are forced to take daily injections of maintenance doses of glucosteroid drugs throughout their lives.

Children should take hormonal medications simultaneously with calcium and vitamin D. The effectiveness of treatment increases due to the use of immunosuppressive drugs from the first days of treatment. These drugs have a depressing effect on the activity of the body's immune system.

Early stages of treatment often involve the use of procedures aimed at clearing the blood of aggressive antibodies. Such procedures include hemodialysis and plasmapheresis. The skin must be treated with antiseptics and special ointments to reduce the risk of infection.

Treatment of pathology is daily and lifelong. Sometimes there may be breaks between relapses of the disease.

Baby care

The diagnosis of pemphigus requires special, attentive and daily care for the child. The organization of a baby’s life directly affects his life expectancy. The initial stage of treatment takes place within the walls of the hospital. After discharge, the child should receive all necessary medications in the prescribed volume and time of administration. Parents must learn how to give injections, because the daily services of a hired nurse will negatively affect the family budget.

Every day your child needs to treat blisters and eczema on the skin. Processing is carried out using aniline dyes. This group of agents has the widest spectrum of action on various microbes, including staphylococcus. Erosion and crusts formed are treated with corticosteroid-based ointments.

The presence of infectious signs in the form of pus, inflammation, swelling means the start of using antibiotic ointments. Large affected areas require the use of sterile dressings to help avoid further injury. Dressings are changed at least 2 times a day. For small affected areas, the relevance of bandages is associated with the child’s high motor activity.

The appearance of pain requires taking painkillers and consulting a specialist. The presence of damaged areas in the oral cavity is associated with mandatory rinsing of the mouth with antiseptic agents. A useful and effective means of care is taking baths with antiseptic agents. It is mandatory to take vitamin and mineral complexes consisting of vitamin E, calcium, magnesium and folic acid.

The diet also requires enrichment with vitamins and minerals. Meals are provided on a fractional and frequent basis, at least 6 times a day. This diet plan is especially important when the esophagus and mucous membranes in the mouth are affected. Diet therapy is based on the complete exclusion of salt and increasing the amount of protein. Registration at a dispensary and systematic visits to a dermatologist are required - at least twice a year and urgently in case of relapse. The use of immunosuppressive therapy imposes restrictions on vaccinations.

Clinical case of pemphigus vulgaris in old age

True pemphigus is one of the most serious diseases. It accounts for 0.7 to 1% of all skin diseases [1, 4]. According to the regional dermatovenerological dispensary in Astrakhan for 2014, only 9 patients with pemphigus were treated as inpatients. Pemphigus can occur at any age. Most often, women over 40 years of age are affected; in recent years, cases of the disease among young people aged 18 to 25 have become more frequent. The most severe course is observed between the ages of 30 and 45 years [1, 4]. Pemphigus (acantholytic, or true, pemphigus) is an autoimmune disease characterized by the appearance of intraepidermal blisters on apparently unchanged skin and/or mucous membranes. The characteristic morphological basis is suprabasal blisters with acantholysis [4, 7, 8]. The etiology of pemphigus still remains unknown [1]. Currently, the leading role of autoimmune processes that develop in response to changes in the antigenic structure of epidermal cells under the influence of various damaging agents is recognized. Cell damage is possible as a result of chemical, physical, and biological factors [4]. It was found that in pemphigus, autoantibodies are directed against the surface structures of epidermal cells - keratinocytes. The binding of autoantibodies (pemphigus IgG) to glycoproteins of cell membranes (pemphigus antigens) of keratinocytes leads to acantholysis - impaired adhesion between cells and the formation of blisters. It has been shown that the complement system and inflammatory cells are not involved in this process, although the presence of complement enhances the pathogenicity of autoantibodies, and infection in areas of skin damage leads to the addition of an inflammatory process, which aggravates the patient’s condition [6]. Risk factors for the development of true pemphigus may include various exogenous and endogenous factors (including genetic predisposition). HLA-DR and HLA-DQ polymorphisms have been shown to be the basis of genetic predisposition to pemphigus (and other autoimmune diseases) [4, 6].

There are four clinical forms of true pemphigus: vulgar (ordinary), vegetative, foliate and erythematous (seborrheic). All clinical varieties are characterized by a long-term chronic wave-like course, leading in the absence of treatment to a disturbance in the general condition of the patients. Pemphigus vulgaris is the most common (up to 80% of all cases) [4]. In more than 50% of cases, the disease begins with damage to the mucous membranes of the oral cavity and pharynx. Small flabby blisters with serous contents that appear on unchanged mucous membranes, initially single or few in number, can be located in any area. Over time, their number increases. The blisters quickly (within 1–2 days) open, forming weeping, painful erosions with a bright red bottom or erosions covered with a whitish coating, bordered along the periphery by fragments of whitish epithelium. With further intensification of the erosion process, they become numerous, increase in size and, merging with each other, form foci of scalloped outlines. Patients experience pain when eating, talking, or swallowing saliva. A characteristic symptom is hypersalivation and a specific putrid odor from the mouth. If the larynx and pharynx are affected, the voice may be hoarse. For a long time, patients are observed by dentists or ENT doctors for stomatitis, gingivitis, rhinitis, laryngitis, etc. Damage to the mucous membranes can remain isolated from several days to 3–6 months. and more, and then the skin of the torso, limbs, and scalp is involved in the process.

Skin damage begins with the appearance of single blisters, then their number increases. The blisters are located on an unchanged, less often on an erythematous background. They are small in size, have a tense tire and serous contents. After a few days, some blisters on the skin dry out into yellowish crusts, or when the tire ruptures, bright red erosions may be exposed, releasing a thick exudate. Erosions at this stage of the disease are not painful and quickly epithelialize. The general condition of the patients remains satisfactory. The rashes that have regressed are replaced by new ones. This initial phase can last from 2-3 weeks. up to several months or even years. Then comes the generalization of the process, characterized by the rapid spread of rashes over the skin and the transition to the mucous membranes of the oral cavity and genitals, if they were not previously affected. As a result of eccentric growth due to exfoliation of the upper layers of the epidermis, the blisters increase in size, the tire becomes flabby, and the contents become cloudy or purulent. Under the weight of exudate, large blisters can take on a pear-shaped form (“Sheklakov’s pear syndrome”). The blisters spontaneously burst with the formation of extensive eroded areas of the skin. Erosions in pemphigus vulgaris are usually bright pink in color with a shiny, moist surface. The peculiarity of erosions is a tendency to peripheral growth, while generalization of the skin process with the formation of extensive lesions, deterioration of the general condition, the addition of a secondary infection, the development of intoxication and, in the absence of treatment, death are possible [1–5]. An important diagnostic sign of pemphigus vulgaris is Nikolsky’s symptom: detachment of the apparently unchanged epidermis when pressing on its surface near the bubble or even on apparently healthy skin far from the lesion [1]. There are three variants of Nikolsky's symptom, which allow us to assess the prevalence of acantholysis. In the first case, when the tire of a burst bladder is pulled, the epidermis peels off beyond its borders. In the second option, the upper layer of the epidermis peels off, and an erosive surface is formed if healthy skin is rubbed between two blisters. The appearance of erosion after rubbing healthy skin in a place near which there are no bullous elements indicates the presence of the third variant of Nikolsky’s symptom [5]. A modification of Nikolsky's symptom is the Asbo-Hansen phenomenon: finger pressure on the tire of an unopened bladder increases its area due to further stratification of the acantholytically altered epidermis with vesical fluid. In the initial phase of pemphigus vulgaris, Nikolsky’s symptom is not always detected, and even then only in the form of a marginal one. When the process is generalized, it is positive in all patients in all modifications [3]. Diagnosis of true pemphigus is based on the totality of the results of clinical, cytological, histological and immunological examination. The clinical picture of the disease, the presence of a positive Nikolsky symptom and its modification, the “pear” phenomenon described by N.D. are taken into account. Sheklakov, which are based on the phenomenon of acantholysis. Cytological examination reveals acantholytic cells (Tzanck cells) in impression smears from erosions and blisters after staining using the Romanowsky-Giemsa method (Tzanck test). The presence of Tzanck cells in the blisters is not pathognomonic, but a very important diagnostic sign of the disease.

Histological examination reveals the intraepidermal location of cracks and blisters [1, 4]. A necessary condition for a qualified diagnosis of true pemphigus is an immunofluorescence study. By means of indirect immunofluorescence, antibodies against components of the epidermis are detected when treated with luminescent anti-IgG human serum. Using direct immunofluorescence, IgG antibodies are detected in skin sections, localized in the intercellular spaces of the spinous layer of the epidermis [1]. Laboratory data (anemia, leukocytosis, increased ESR, proteinuria, hypoalbuminemia, decreased sodium excretion in urine, etc.) play a certain supporting role, allowing one to assess the severity of the process [3]. Differential diagnosis is carried out with Lever's bullous pemphigoid, Dühring's dermatitis herpetiformis, chronic benign familial pemphigus Gougereau-Hailey-Hailey, lupus erythematosus, seborrheic dermatitis, Lyell's syndrome, chronic pyoderma vegetans [4].

Treatment of true pemphigus has so far caused great difficulties. Since the main links of pathogenesis are interpreted from the standpoint of autoimmune pathology, all existing therapeutic measures are reduced to immunosuppressive effects on autoallergic processes through the use of corticosteroid and cytostatic drugs [1]. The introduction of corticosteroids into the treatment of pemphigus reduced mortality among patients from 90 to 10% [6]. Pemphigus is one of the few diseases in which corticosteroids are prescribed for health reasons, and existing contraindications in these cases become relative. The positive effect of glucocorticoids is explained primarily by the blockade of key stages of the biosynthesis of nucleic acids and proteins, shutdown of the afferent phase of immunogenesis, reduction of lymphoid organs, destruction of medium and small thymic lymphocytes, and inhibition of the formation of immune complexes. It is also believed that corticosteroids have a stabilizing effect on lysosome membranes and inhibit the synthesis of autoantibodies [1]. Typically, pemphigus vulgaris and pemphigus foliaceus are the most severe, therefore, for these clinical forms, the highest doses of glucocorticosteroids are prescribed (from 60–100 to 150–300 mg/day of prednisolone equivalent) [1,4]. The dose of prednisolone is selected taking into account the prevalence of the rash and the severity of the disease. It should be at least 1 mg/kg/day. The daily dose is distributed in such a way that 2/3 falls in the early morning hours (preferably after meals), and 1/3 in the afternoon (12–13 hours). If the patient’s condition is particularly severe, higher doses of prednisolone are prescribed – up to 300 mg/day [4]. At high doses, prednisolone can be partially replaced by parenteral administration or betamethasone (it is possible to use prolonged forms once every 7-10 days). Long-term use of corticosteroid drugs leads to the development of serious complications and side effects, and if they are quickly discontinued, the so-called withdrawal syndrome occurs, and the disease recurs. Therefore, it is necessary to correct and prevent side effects caused by long-term use of glucocorticosteroids. In order to prevent withdrawal syndrome, it is recommended to stop taking medications or reduce their daily dose carefully and gradually. Initially, a reduction in the dose of glucocorticosteroids is possible by 1/4–1/3 of the maximum initial dose after achieving a clear therapeutic effect (cessation of the appearance of new blisters, active epithelization of erosions), which usually occurs after 2–3, sometimes after 4 weeks. Then the dose of prednisolone is gradually, slowly, over several months, reduced to maintenance. The daily dose of the hormone is gradually reduced, approximately once every 4–5 days by 2.5–5 mg of prednisolone until the minimum maintenance effective dose of the corticosteroid is reached, the administration of which ensures remission of the disease.

In the future, a maintenance dose of corticosteroids is recommended to be administered alternately. However, periodically (every 4–6 months) it should be reduced by 2.5 mg prednisolone equivalent. Thus, by reducing the maintenance dose, it is possible to reduce the amount of hormone administered by 3-4 times compared to the initial maintenance dose. The maximum permissible minimum maintenance dose can vary from 2.5 to 30 mg/day. Typically, patients with pemphigus receive glucocorticosteroids for life, and sometimes their use can be abandoned [1, 4]. The addition of second-line drugs to therapy is indicated to increase the effect of treatment, reduce the side effects of corticosteroids, and also to prevent relapses during their gradual withdrawal. Adjuvant therapy includes azathioprine, methotrexate, cyclophosphamide, mycophenolate mofetil, intravenous immunoglobulin and dapsone [8]. The combined use of cytostatic and immunosuppressive drugs with corticosteroids allows one to achieve good therapeutic results in a shorter time and with lower daily doses of corticosteroids. Many drugs, such as alkylating agents and antimetabolites, have cytostatic properties. Of the alkylating agents, cyclophosphamide is the most widely used in the treatment of pemphigus. This drug is capable of entering into alkylation reactions with certain groups of proteins and nucleic acids of the cell, inhibiting various enzyme systems and dramatically disrupting the vital activity of cells, primarily highly active and lymphoid ones. Antimetabolites, which include purine base antagonists (azathioprine) and folic acid antagonists (methotrexate), resemble the structure of natural cell metabolites and, by competing with them, disrupt intracellular metabolism. The consequence of this is the accumulation of substances toxic to cells, leading to the death of cells, primarily actively proliferating ones. Azathioprine is prescribed at a dose of 1.5–2 mg/kg/day in 2–4 divided doses in combination with steroids. Methotrexate is administered intramuscularly at 10–20 mg (with good tolerance up to 25–30 mg) 1 time per week (for a course of 3–5–8 injections). Cyclophosphamide is administered orally at 100–200 mg/day, the duration of therapy is determined individually. During treatment, monitoring of blood tests (general and biochemical) and urine is necessary.

If the therapeutic effectiveness of glucocorticosteroids is insufficient and there are contraindications to the use of cytostatics, immunosuppressants are prescribed. Cyclosporine A for the treatment of patients with true pemphigus is used in combination with corticosteroid drugs, and the daily dose of corticosteroids is reduced by 3-4 times and corresponds to 25-50 mg of prednisolone equivalent. The daily dose of cyclosporine A in the complex therapy of patients with true pemphigus in the acute stage should not exceed 5 mg per 1 kg of the patient’s body weight and averages 3–5 mg/kg/day. This takes into account the clinical picture, severity and prevalence of the disease, the patient’s age, and the presence of concomitant diseases. For the first 2 days, to assess the tolerability of the drug, cyclosporine A is prescribed in half the dose, subsequently the daily dose is divided into 2 doses - morning and evening with an interval of 12 hours. The daily dose of cyclosporine A begins to be reduced after intensive epithelization of existing erosions. Typically, a loading dose is taken on average for 14–20 days, followed by a gradual reduction in the daily dose of the drug to 2–2.5 mg per 1 kg of the patient’s body weight. Complete cleansing of the skin should not be considered the final goal of treatment. After achieving remission, the patient should continue to take the minimum effective maintenance dose of cyclosporine A, which should be selected individually. At this dosage, the drug can be used for a long time (2–4 months) as maintenance therapy. Currently, treatment with immunosuppressants is not generally accepted [1, 4]. Aniline dyes, corticosteroid creams with an antibacterial or antimycotic component, and aerosols are used locally [3]. For additional treatment of pemphigus, extracorporeal detoxification methods (hemosorption, plasmapheresis) are successfully used [1]. Despite the successes of domestic and foreign researchers in clarifying the mechanisms of pathogenesis and improving treatment methods for patients with true pemphigus, the problem of pemphigus remains relevant and is due to the severity of the disease, its incurability and potential mortality [1].

We present a clinical case demonstrating the difficulties of differential diagnostic search when diagnosing pemphigus. Patient Zh., born in 1945, has been ill since the fall of 2014, when rashes first appeared on the scalp. I treated myself, used ointments with antibiotics without effect. In May 2015, an exacerbation of the skin process began. The rash has spread to the face and torso. He was observed in the clinic at his place of residence, and due to the torpidity of treatment, he was sent to the regional oncology clinic, where a pathohistological examination of the scalp was carried out. After examination, a diagnosis was made: multiple scalp cancer. Т1N0М0. Radiation therapy is recommended. Within 2 weeks. the process on the skin has spread significantly: elements have appeared on the chest, back, and upper limbs. Serous-purulent discharge was noted on the scalp, and the erosions took on a draining character. Taking into account the change in the clinical picture, he was sent for consultation to the regional dermatovenerological clinic with a diagnosis of pemphigus vulgaris. As a result of examination in the department, this diagnosis was clinically and laboratory confirmed. At the same time, pathohistological preparations were reviewed at the oncology clinic, and the diagnoses of cancer and basal cell carcinoma of the scalp were removed as erroneous. From the anamnesis it is known that the patient has a burdened premorbid background. In 2012, he suffered a small-focal myocardial infarction. Suffering from chronic bronchitis. Upon examination, a widespread pathological process was revealed. On the skin of the scalp, face, neck, torso and upper extremities there were multiple bright red erosions with serous and serous-purulent discharge. Some of the erosions were covered with dense crusts of gray-yellow color (Fig. 1–3). On an erythematous background, there were blisters of various sizes with a flabby cap and cloudy contents. Nikolsky's symptom is positive. During a laboratory examination, smears from blisters on the forearms revealed single acantholytic cells in a specimen with coarse nuclei. Epithelial cells with signs of atypia (enlarged coarse nuclei, binucleate cells) up to 8–10–12 per field of view were identified. During bacteriological examination of discharge from erosions, staphylococcus was isolated. According to the pathomorphological study, the morphological picture of pemphigus was revealed - a bubble with serous fluid and acantholytic cysts with purulent inflammation along the periphery was found in the epidermis. When reviewing the drugs at the oncology center, the morphological picture of pemphigus was confirmed and no tumor growth was detected. A general clinical examination showed an increase in ESR and a sharply positive C-reactive protein. Fibrogastroduodenoscopy revealed erosive antrum gastritis, erosive and ulcerative bulbitis and duodenitis. Small (up to 0.2 cm) acute ulcers of the bulb and the upper horizontal part of the bulb were found.

Taking into account the generalized process of skin lesions, parenteral administration of prednisolone at a dose of 90 mg was prescribed with a gradual transition to oral administration of the drug at a dose of 30 mg. He also received detoxification, antibacterial and antifungal systemic therapy and local treatment using antiseptics, reparatives and anti-inflammatory drugs. Due to the presence of erosive gastritis, erosive-ulcerative bulbitis, and also because of taking prednisolone, the patient was given antisecretory therapy. During therapy for 3 weeks. positive dynamics were noted. On the skin of the scalp, neck, chest, and back, erosions were completely epithelialized, and the crusts fell off (Fig. 4–6). He was discharged for outpatient treatment with recommendations to reduce the dose of prednisolone by 1/4 tablet every 7–10 days under the supervision of a dermatovenerologist at the place of residence.

The development of pemphigus in a 70-year-old man with a latent onset of the disease may have been the reason for late diagnosis and, consequently, untimely initiation of therapy. After correct interpretation of clinical and morphological data and complex therapy, a positive result was achieved.

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