Diagnostic signs of dermatological diseases. True pemphigus (acantholytic) or pemphigus: characteristics of the disease and treatment methods. Examination of scales, hair, nails for pathogenic fungi

True or acantholytic pemphigus- bullous dermatosis, characterized by the formation of intraepithelial blisters on unchanged skin and/or mucous membranes as a result of acantholysis.

The disease has a long history chronic course with remissions varying degrees severity and varying duration.

There are 4 clinical forms of acantholytic pemphigus:

• vulgar,
• vegetative,
• leaf-shaped
• seborrheic (erythematous).

What provokes Pemphigus vera (acantholytic):

Etiology unknown. There are viral and autoimmune theories of this disease. Currently, the leading role of autoimmune processes in the pathogenesis of true (acantholytic) pemphigus has been proven.

Pemphigus vulgaris, or vulgar, occurs much more often than other forms. Mostly men and women from 40 to 60 years old are affected; in childhood and adolescence it is very rare.

Symptoms of true pemphigus (acantholytic):

Most often, the oral mucosa is affected by ordinary (vulgar) pemphigus (in 75% of patients), so it is of greatest interest to dentists. Pemphigus vulgaris almost always begins with damage to the mucous membrane of the mouth or larynx, and then spreads to the skin. Even if the disease begins with skin lesions, lesions of the oral mucosa are almost always detected later.

Damage to the mucous membrane of the mouth and lips with pemphigus is characterized by the formation of single blisters with serous or hemorrhagic contents, having a very thin covering. Due to constant maceration in the oral cavity, the blisters open very quickly, so they are rarely seen. Along the periphery of the erosion, fragments of bubble tires are often found. In place of the blisters, painful erosions of a round, oval or elongated crack-like shape are formed, which do not heal for a long time. Erosions of bright red color are located against the background of unchanged or slightly inflamed mucous membrane. Their sizes in pemphigus vary - from a small abrasion to large surfaces of congestive red color. As a rule, there is no plaque on the surface of erosions, or there may be a thin layer of easily removable fibrinous plaque. Sometimes, instead of bubbles, white (greasy) films are formed, after which, when rejected, an erosive surface is exposed. With the progressive course of the disease due to the appearance of new blisters and pronounced acantholysis, the number of erosions and their size increase. When erosions merge, extensive lesions are formed, covering almost the entire mucous membrane of the mouth. Hypersalivation is possible. Most often, erosions are localized on the mucous membrane of the cheeks (especially in the retromolar region), the lower surface of the tongue, palate and the floor of the mouth. Sometimes lesions occur on the mucous membrane of the alveolar processes, transitional fold, lower and upper lips. In these cases, erosions epithelialize very slowly, even while taking large doses of corticosteroids.

In the absence of treatment, new erosions appear, which, merging with each other, form extensive erosive surfaces without a tendency to heal. The pain is quite severe, most intense when eating and talking. Erosions quickly become infected, especially in an unsanitized oral cavity. The addition of coccal, fungal flora and fusospirochetosis aggravates the patient’s condition, causing a specific foul odor from the mouth. Salivation intensifies. Saliva macerates the corners of the mouth, painful cracks appear. On the red border of the lips, in the corners of the mouth, blisters and erosions covered with hemorrhagic crusts are also possible. Sometimes hoarseness occurs, indicating damage to the larynx.

On the skin, blisters form mainly in places of friction with clothing (stomach, back, groin folds, etc.). After opening the blisters, very painful erosions remain on the skin. Any touch of clothing, linen or bandages to them causes sharp pain and forces the patient to remain motionless for hours.

Nikolsky's symptom in pemphigus is usually positive. There are three varieties of it:

• if you grab the lid of the bladder or the upper layer of the epithelium at the edge of the erosion with tweezers and pull, then 37 Zak. 5491. Yu. M. Maksimovsky describes the detachment of the epithelial film on the apparently unchanged healthy mucous membrane and skin. The thin film of epithelium is very fragile and is easily cut with tweezers;
• rubbing the unchanged mucous membrane or skin between the affected areas leads to the rapid formation of blisters or erosions;
• if you rub areas located far from the affected area, the upper layers of the epithelium there will also peel off. The second and especially the third types of Nikolsky’s symptom indicate an increase in the intensity of acantholysis.

With pemphigus, in addition to the skin and mucous membrane of the mouth, other mucous membranes (intestines, stomach, esophagus, pharynx), as well as internal organs and the central nervous system can be affected.

Pemphigus is characterized by an undulating course, periods of exacerbation are followed by periods of remission, which rarely occur spontaneously, usually after treatment. In the absence of timely and necessary treatment the disease is steadily progressing. Rapid generalization of rashes on the skin and oral mucosa is possible, the general condition of patients worsens, weakness, malaise, loss of appetite, fever up to 38-39 ° C, and diarrhea appear; swelling of the lower extremities. The addition of a secondary infection is accompanied by cachexia and intoxication. Without treatment, the process ends in death several months after the onset of the disease.

However, due to widespread use corticosteroids, such cases are now rare. Corticosteroid therapy interrupts the progressive course of pemphigus, and a stage of remission occurs.

Dühring's dermatitis herpetiformis characterized by a subepithelial arrangement of blisters. The blisters are small, tense, located on a swollen, hyperemic background, tend to merge, their formation is accompanied by burning and itching. Unlike pemphigus, rashes with Dühring's dermatitis are very rarely localized on the mucous membranes. Nikolsky's sign is negative, acantholytic cells are absent. The blisters contain a significant content of eosinophils, and there is also eosinophilia in the blood. In patients dermatitis herpetiformis Dühring's sensitivity to iodine is often increased.

In some cases, with pemphigoid, red lichen planus and other blistering diseases, when the blisters are subepithelial, around them or erosions the upper layer of the adjacent epithelium can quite easily peel off. At the same time, the bladder cover is thick and difficult to rupture. This symptom is called false symptom Nikolsky, or a symptom of perifocal subepithelial detachment.

Benign non-acantholytic pemphigus of the oral mucosa differs from ordinary pemphigus by the localization of blisters only on the oral mucosa, subepithelial location, dense covering of blisters, often with hemorrhagic contents, and the absence of acantholytic cells.

Diagnosis of true pemphigus (acantholytic):

Diagnosis diagnosed based on clinical manifestations, positive Nikolsky sign, results cytological examination and direct immunofluorescence reaction.

Cytological examination of smear impressions or scrapings from the bottom of erosions is mandatory for the diagnosis of pemphigus. The presence of acantholytic cells in them confirms the diagnosis of acantholytic pemphigus. Acantholytic cells, or Tzanck cells, which are modified cells of the spinous layer, have a round outline and are smaller in size than normal cells of the spinous layer. The nucleus is large relative to the entire cell, its diameter is y - y or more than the diameter of the cell, colored dark blue, often has from 1 to 6 light nucleoli or more. The cytoplasm of cells is heterogeneously colored: light blue around the nucleus and dark blue along the periphery. Acantholytic cells are characterized by polymorphism in color, size of cells and nuclei. There are giant multinucleated cells - “monsters”. At the height of the disease, the number of acantholytic and multinucleated cells increases sharply. They merge into a continuous conglomerate of polymorphic cells. During remission and during treatment with corticosteroids, the number of acantholytic cells decreases.

The cytological picture of pemphigus vegetans does not differ from that of pemphigus vulgaris. In seborrheic pemphigus, multinucleated cells, as a rule, are not found; acantholytic cells are found in smaller numbers, they are monomorphic.

Pathohistological studies. It has been established that the main morphological changes in acantholytic pemphigus are acantholysis and edema, resulting in the formation of intraepithelial blisters. The connections between the cells of the spinous layer are disrupted - the phenomenon of acantholysis, as a result of which the intercellular bridges melt, gaps and then bubbles form between the cells. The bottom of such blisters, as well as subsequently the surface of erosions, is lined predominantly with acantholytic cells.

• Differential diagnosis

Acantholytic (true) pemphigus must be differentiated from other bullous lesions of the oral mucosa:

• exudative erythema multiforme;
• pemphigoid;
• drug allergies;
• bullous form of lichen planus;
• herpeti for a lot of Dühring's dermatitis;
• benign non-acantholytic pemphigus of the oral mucosa only.

Differential diagnosis of acantholytic pemphigus with other diseases accompanied by the formation of blisters is based mainly on the location of the blisters in relation to the epithelium.

Thus, with exudative erythema multiforme, the blisters are surrounded by a zone of erythema along the periphery, located subepithelial, Nikolsky’s symptom is negative. In addition, exudative erythema multiforme is characterized by an acute onset, seasonal relapses, severe inflammation of the oral mucosa, and a short course.

With bullous pemphigoid, the blisters are located under the epithelium, their cover is thick, so their existence is longer. Bullous pemphigoid most often affects people over 60 years of age. Nikolsky's sign is negative, no acantholytic cells are detected.

Anamnesis (information about taking medications) and the results of allergy tests help to distinguish acantholigic pemphigus from allergic drug stomatitis. After discontinuation of the allergen drug, stomatitis quickly disappears. Bubbles when medicinal stomatitis are located under the epithelium, Nikolsky's symptom is negative, there are no acantholytic cells.

In the bullous form of lichen planus, the blisters are subepithelial, there is no acantholysis. There are multiple papules around the blisters or other areas of the oral mucosa, typical of lichen planus.

Treatment of Pemphigus vera (acantholytic):

Treatment of pemphigus currently includes corticosteroids, which are the mainstay of treatment for this disease. Other medications, including cytostatics, are used to eliminate complications associated with taking glucocorticoids. The success of treatment with glucocorticoids depends on the timing of their use and dosages. The more correctly the doses of corticosteroid drugs are selected and the earlier their use is started, the greater the opportunity to achieve stable and long-term remission of the disease. Treatment of patients with acantholytic pemphigus should be carried out only in a specialized hospital.

For the treatment of patients with pemphigus, prednisolone, methylprednisolone (Metypred, Urbazone), dexamethasone (Dexazone), triamcinolone (Polcortolone, Kenacort) are prescribed in loading doses, which depend on the patient’s condition. Prednisolone is prescribed at 60-80 (up to 100) mg/day, triamcinolone at 40-80 mg/day, dexamethasone at 8-10 mg/day. Patients take such high, so-called loading doses until the formation of new blisters stops and erosions are almost completely epithelialized, which is an average of 10-15 days. After which the daily dose of prednisolone is slowly reduced, first by 5 mg every 5 days, later these periods are extended to 7-10 days. When the daily dose reaches 20-30 mg, it is reduced very carefully. Subsequently, the daily dose is reduced until the minimum, so-called individual maintenance daily dose, which is administered permanently, is determined. For prednisolone it is usually 2.5-5 mg, for dexamethasone - 0.5-1 mg, every 4-5 days.

Long-term use of corticosteroid drugs causes side effects (increased blood pressure and glucose in the urine, osteoporosis bone tissue, increased thrombus formation, etc.). In this regard, to reduce complications from corticosteroid therapy, it is recommended to limit the intake of table salt and water. The diet should be predominantly protein with limited fat and carbohydrates. Potassium preparations are prescribed orally (potassium chloride, asparkam), ascorbic acid, B vitamins, calcium supplements, thyrocalcitonin.

Along with glucocorticoids, immunosuppressants are used in the treatment of patients with acantholytic pemphigus.

For the treatment of patients with acantholytic pemphigus use plasmapheresis and hemosorption, which help reduce side effect glucocorticoids and cytostatics, and also allow you to reduce their dose.

Local treatment , mainly aimed at preventing secondary infection of erosions and ulcers and accelerating their epithelization, includes painkillers in the form of oral baths; antiseptic drugs in non-irritating concentrations; applications to the oral mucosa or lubrication with corticosteroid ointments. After each meal and before applying corticosteroid-containing ointments, rinsing with warm weak solutions of potassium permanganate, 0.25% chloramine, 0.02% chlorhexidine, etc. is necessary. Thorough sanitation of the oral cavity is important for the rapid epithelization of erosions on the mucous membrane. If the red border of the lips is affected, applications and lubrication are carried out with ointments containing corticosteroids and antibiotics, as well as oil solution vitamin A. When pemphigus is complicated by candidiasis, antifungal drugs are prescribed. To accelerate the epithelization of erosions and ulcers on the oral mucosa, laser therapy (helium-neon and infrared laser) is indicated.

However, even with the correct and timely treatment The prognosis for true (acantholytic) pemphigus remains serious. Patients who take corticosteroid drugs for a long time need sanatorium treatment (gastrointestinal and cardiovascular profile). Insolation is strictly contraindicated for them.

Pemphigus (Pemphygus; synonym: true pemphigus, acantholytic) – autoimmune disease, characterized by the formation of intraepidermal blisters that form as a result of acantholysis.

Characterized by generalization of rashes and a steadily progressive course, which ends in death within a period of 6 months to 1.5-2 years (deviations are possible, sometimes very significant, in both directions). The increasing severity of the disease can be interrupted by remissions of varying severity and duration. Pemphigus usually affects people aged 40-60 years, mainly women, but it is possible that any person can be affected. age group.

The following types of pemphigus are distinguished: vulgar, vegetative, leaf-shaped, erythematous. This division is relative: transformation of one form into another is possible, especially against the background of corticosteroid therapy, and a combination of different forms.

Etiology and pathogenesis. Etiology unknown. The most promising theory is that pemphigus is caused by a representative of retroviruses in the presence of a genetic predisposition. The pathogenesis of pemphigus is based on autoimmune processes, the essence of which is the formation of autoantibodies to the cementing intercellular substance and cell membranes of the spinous layer under the influence of changes in their antigenic structure. They by their nature belong to IgG and in the direct immunofluorescence reaction they are detected in the form of fixed antigen-antibody complexes in the intercellular bridges (in the area of ​​​​the junction of desmosomes and tonofilaments) of the epidermis, leading to the destruction of the connection between cells - acantholysis, which is facilitated by the activation of esterase proteolytic systems under action of immune complexes. When performing an indirect immunofluorescence reaction in the cystic fluid and blood serum of patients with pemphigus, “pemphigus-like” autoantibodies are also detected. There is activation of B- and inhibition T cell immunity, decreased synthesis of interleukin-2. A certain place in the pathogenesis of true pemphigus is occupied by disturbances in water and especially salt metabolism, as evidenced by a sharp decrease in the daily excretion of sodium chloride in the urine. The cytological feature of true pemphigus is acantholytic cells (Tzanck cells), formed as a result of loss of communication between keratinocytes and used as a diagnostic test. They are identified in fingerprint preparations: material from the bottom of a fresh erosion with a piece of student gum, sterilized by boiling, is transferred to a glass slide, dried in air, fixed and stained with hematoxylin and eosin. Acantholytic cells of pemphigus, which are usually smaller in size than normal cells, have a very large nucleus of intense purple or violet-blue color, occupying almost the entire cell. It contains two or more light-colored nucleoli. The cytoplasm is sharply basophilic, around the nucleus it is light blue, and along the periphery it is dark purple or blue. Acantholytic cells can be single or multiple, forming clusters or even layers. In the beginning, acantholytic cell diseases are not found in every preparation; at the height of the disease there are many of them. The cytological method facilitates the recognition of pemphigus, especially if there are many acantholytic cells and they are detected repeatedly. An obligate symptom of true pemphigus is acantholysis, leading to the formation of intraepidermal blisters. Histologically, they are revealed in the form of horizontal cracks and cavities, the contents of which include fibrin, neutral leukocytes, sometimes eosinophils and complexes of acantholytic cells. With vulgar and vegetative pemphigus, the cavities are located suprabasally, with foliate and erythematous - in the zone of the granular layer, often under the stratum corneum.

Clinical picture.

Pemphigus vulgaris occurs most often. The disease usually begins with damage to the oral mucosa, which is often facilitated by influenza, sore throat, tooth extraction and prosthetics. It can remain isolated from several days to 3-6 months or more, then the skin becomes involved in the process. Small blisters that appear on the mucous membranes, initially isolated, can be located in any area; Over time, the number of bubbles increases. Their thin and flabby cover, under conditions of maceration and continuous pressure during chewing and moving the tongue, quickly opens, revealing painful bright red or covered with a whitish coating of erosion, bordered along the periphery by scraps of whitish epithelium. With further growth of the erosion process, they become numerous and increase in size; merging with each other, they form extensive lesions with scalloped outlines. Salivation is increased. Eating is difficult or almost impossible due to pain. When the larynx and pharynx are affected, the voice becomes hoarse. On the red border of the lips, erosions are covered with serous, hemorrhagic or impegiginized crusts. Heavy putrid smell from the mouth haunts the patient and those around him. In the remission stage, erosions of the oral mucosa heal without scars. Sometimes initial manifestations diseases are localized on the mucous membranes of the genital organs. The conjunctiva of the eyes is involved secondarily. Defeat skin begins gradually, with the appearance usually of single blisters in the chest and back area. Over time, their number increases. The blisters are located on an unchanged, less often erythematous background; are small in size and have serous contents; after a few days they dry out into yellowish crusts, which fall off, leaving hyperemic spots, or when the bubble is opened, bright red erosions form, releasing a thick exudate. Erosions at this stage are of little pain and quickly epithelialize. The general condition of the patients remains satisfactory. Instead of rashes. regressed, new ones appear. This initial phase can last from 2-3 weeks 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 mouth and genitals, if they were not previously affected. The rashes become profuse, disseminated, and if left untreated can lead to total damage to the skin. As a result of eccentric growth due to peeling upper layers epidermal blisters increase in size, reaching 3-4 cm or more in diameter; can merge with each other; their cover is flabby, and their contents are cloudy. Large blisters, under the weight of the exudate, take on a pear-shaped shape - the “pear symptom”. Tires bubble even when minor injury rupture, which leads to the formation of erosions. Erosion has a bright red or bluish-pink color; covered serous exudate, soft grayish-white or brownish plaques or loose crusts, when forcibly rejected, slight bleeding occurs. Characteristic features of erosions in pemphigus are a tendency to peripheral growth and lack of epithelization. As a result of peripheral growth and fusion, erosions reach large sizes - up to the palm of an adult and more. At places of pressure and friction (shoulder blades, buttocks, large folds) they can occur without prior formation of bubbles. Important feature pemphigus vulgaris, as well as other forms of true pemphigus. is Nikolsky's symptom, the essence of which is mechanical detachment (rejection and shifting of the upper layers) of the epidermis. It is caused by finger friction (sliding pressure) externally healthy skin both near the bubble and at a distance from it, or by pulling a piece of the bladder cover, which leads to detachment of the upper layers of the epidermis in the form of a gradually narrowing band on apparently healthy skin. Its modification is the Asbo-Hansen phenomenon: finger pressure on the cover of an unopened bladder increases its area due to further stratification of the acantholytically altered epidermis with vesical fluid.

With the generalization of skin rashes, there is a deterioration in the well-being and general condition of patients: weakness, malaise, loss of appetite, insomnia, fever up to 38-39°C, diarrhea, swelling, especially of the lower extremities; secondary infections occur, cachexia develops, which is facilitated by difficulty eating as a result of damage to the mucous membranes of the mouth, significant loss protein (plasmorrhea) and intoxication. Without treatment, patients die from secondary infections and cachexia.

Pemphigus vegetans characterized by a predominance of vegetative elements and a more benign course. Bubbles with pemphigus vegetans, which arise initially, as with vulgar pemphigus, most often on the oral mucosa, are then located mainly around natural openings and in skin folds(axillary fossae, groin areas, under the mammary glands, interdigital folds, navel, behind the ears). When the blisters are opened, the sizes of which, as a rule, are smaller than those of pemphigus vulgaris, succulent vegetations of pink-red color, soft consistency, with a height of 0.2 to 1 cm or more are formed on the surface of the erosions; their surface is covered with a grayish coating, serous or purulent discharge, crusts; there is a foul odor. On the skin outside the folds and on the mucous membranes, vegetations are rare; the evolution of blisters in these areas is the same as in pemphigus vulgaris, but at the border of the mucous membranes with the skin (lips, nose, genitals, anus) growing seasons are frequent. During regression, the vegetation dries out, flattens, and erosion undergoes epithelialization, leaving post-eruptive hyperpigmentation. The course of pemphigus vegetans is longer than that of pemphigus vulgaris; there may be complete and long-term (several months or even years) remissions. Nikolsky's symptom is positive only near the lesions. On apparently healthy skin it is usually detected in terminal stage, in which, against the background of increasing deterioration, the skin lesion becomes extremely similar to the manifestations of pemphigus vulgaris.

Pemphigus foliaceus (exfoliative) It is less common than vulgar, but more often vegetative; the mucous membranes, with rare exceptions, are not affected. It is characterized by flat, small bubbles with a thin and flabby covering. They usually occur on an erythematous background. Their tire quickly and easily ruptures even with minor injury or under the influence of increasing pressure from the vesical fluid. The resulting pink-red erosions are superficial, with copious serous discharge, drying into lamellar crusts. Such crusts can form without rupturing the lining of the bubbles due to the drying out of their contents. Thin lamellar peels resemble sheets of paper, which explains the name of this variety. Usually the crusts are not rejected, since the exudate continues to separate underneath them, which leads to the formation of a new layer of crusts. As a result, massive layered crusts are formed. The disease often begins with damage to the skin of the face, scalp, chest and upper back. Sometimes it is limited to these localizations for a long time - months and even years. More often there is a rapid spread of the process throughout the skin. The affected skin is diffusely hyperemic, edematous, covered with flabby blisters, weeping erosions, scales, and layered crusts. Nikolsky's symptom is expressed sharply, including apparently healthy skin. Flow pemphigus foliaceus can be long-term – up to 2-5 years or more. The general condition of patients may remain satisfactory for many months, and sometimes even years, but general weakness and malaise gradually increase, and death occurs.

Pemphigus erythematous (seborrheic) It begins, as a rule, with damage to the face or scalp, followed by spread to the chest, interscapular area, large folds and other areas of the skin. The mucous membranes and conjunctiva of the eyes are rarely involved. The initial rashes are represented by pink-red plaques with a diameter of 2 to 5 cm, with clear boundaries, rounded and irregular outlines. Their surface may be covered with white, dry, tightly packed scales, which gives these rashes a resemblance to lupus erythematosus lesions. More often, the surface of the plaques is covered with fatty yellowish-brown scales and crusts, making them similar to the manifestations of seborrheic eczema, especially in the case of weeping and erosion. Nikolsky's symptom is positive or more often weakly positive, marginal. Over time, from 2–3 weeks to 2–3 years or more, blisters appear, similar to those in pemphigus vulgaris and pemphigus foliaceus. They begin to prevail in the clinical picture of the disease, which leads to the transformation of erythematous pemphigus into vulgar or, more often, foliaceous.

The diagnosis of true pemphigus is based on clinical symptoms, the nature of Nikolsky’s symptom, the results of cytological (Tzanck cells), histological (intraepidermal blisters) and immunofluorescent studies (fixation of IgG in the intercellular substance of the epidermis and detection of circulating “pemphigus-like” autoantibodies in the blood). Differential diagnosis carried out with Lever's bullous pemphigoid, Dühring's dermatitis herpetiformis, seborrheic eczema, chronic ulcerative-vegetative pyoderma.

Treatment: the main drugs are corticosteroid hormones. An initial dose of prednisolone from 80 to 100 mg/day is usually sufficient to stop the process. Sometimes, however, higher doses are required (up to 200 mg/day or more). With an adequately selected daily dose, a clear therapeutic effect occurs within 10-14 days. Reducing the daily dose, especially high at the beginning, is possible immediately by 1/4-1/3 of the initial one when stopping the process; for the next 2 weeks, the dose, as a rule, is not changed; further reduction is carried out slowly to the minimum maintenance level. Once the daily dose of 20-30 mg is reached, further reduction should be done with great caution to avoid relapse of the disease. With this approach, the maintenance dose can be 5.0-2.5 mg. In addition to prednisolone, triamcinolone (kenacort, polcortolone), methylprednisolone, metypred, urbazone, dexamethasone, betamethasone are used for pemphigus in doses equivalent in effect to prednisolone.

Corticosteroid therapy, which usually lasts for a long time, sometimes for many years, is inevitably accompanied by a variety of complications, including the Itsenko-Cushing symptom complex, obesity, steroid diabetes, erosive and ulcerative pathology digestive tract, hypertension, thrombosis and thromboembolism, osteoporosis leading to spinal fracture, hemorrhagic pancreatitis, insomnia, euphoria, depression, acute psychosis, myocardial infarction, cerebral stroke, as well as the addition of various infections. In order to prevent complications, a diet rich in proteins and vitamins, with a sharp limitation of carbohydrates, fats and table salt, is recommended; taking potassium chloride up to 3 g per day; protectors of the gastric mucosa, as well as anabolic hormones, B vitamins, and when a secondary infection occurs - antibiotics and anticandidal agents. As an adjunct to corticosteroid therapy, especially in severe forms pemphigus, methotrexate, azathioprine and cyclophosphamide are prescribed, the immunosuppressive effects of which are well known. Methotrexate is administered intramuscularly at 25 mg at intervals of 1 week; for a course of 6-8 injections. The number of courses and the intervals between them are determined by the severity of the disease. Azathioprine and cyclophosphamide are prescribed orally at 50-250 mg (2.5 mg per 1 kg of body weight) and 100-200 mg per day, respectively. The duration of use of cytostatics depends on the therapeutic effect and their tolerability. To the most serious complications Treatment with cytostatics includes ulceration of the mucous membrane of the digestive tract, dysfunction of the liver, kidneys, pancreas, hematopoietic disorders, complications of microbial, mycotic, viral infections, disorders of spermato- and oogenesis, alopecia. In order to remove circulating autoantibodies from the body and increase sensitivity to corticosteroid hormones, they are used, especially in initial stages diseases, extracorporeal treatment methods: plasmapheresis, hemosorption and hemodialysis. In case of pronounced inhibition of T-cell immunity, tactivin is prescribed 100 mcg subcutaneously every other day (No. 10), then 100 mcg every 15 days for 2-4 months. Local treatment for pemphigus plays a supporting role. Solutions of aniline dyes, corticosteroid ointments (celestoderm V with garamycin, hyoxysone, etc.), 5% dermatol or xeroform ointment are used. The prognosis for pemphigus is always serious; prevention of relapses, in addition to rational treatment, includes a gentle general regimen, exclusion of colds and intense insolation. Patients with pemphigus should be under constant medical supervision.

Bullous pemphigoid (syn. neacantholytic pemphigus, Lever's bullous pemphigoid) – benign chronic illness skin, the primary element of which is a bubble that forms subepidermally without signs of acantholysis. In this regard, acantholytic cells are not detected, Nikolsky’s symptom is negative.

Etiology and pathogenesis. The etiology of the disease is unknown. Due to the fact that dermatosis in some cases can be of a paraneoplastic nature, all elderly patients with bullous pemphigoid undergo an oncological examination to exclude cancer internal organs. Allowed viral etiology dermatosis. The autoallergic pathogenesis of the disease is most substantiated: autoantibodies (usually IgG, less often IgA and other classes) to the basement membrane of the epidermis, both circulating in the blood and vesical fluid, and fixed at the sites of blistering, have been found. Circulating and fixed antibodies are detected in indirect and direct immunofluorescence reactions, respectively. It is assumed that during the formation of blisters, autoantibodies in the basement membrane area bind to antigens and activate complement. This process is accompanied by damage to the cells of the basal layer. Lysosomal enzymes inflammatory cells, attracted to the site of action under the influence of the chemotactic activity of complement components, enhance destructive processes in the area of ​​the basement membrane, which ends in the formation of a bubble.

The earliest histological feature of pemphigoid is the formation of subepidermal microvacuoles. Their fusion leads to the formation of blisters that separate the epidermis from the dermis, sometimes microvacuoles remain around the blisters. Fresh bubbles are round and small in size; intercellular lacunae are expanded, but without signs of acantholysis. Soon after the formation of blisters, re-epithelialization of their bottom occurs.

Electron microscopy revealed that bubbles arise in the lamina pellucida, the zone between the basal cell plasma membrane and the basal lamina. In the dermis there is an infiltrate of neutrophils, eosinophils and histiocytes. Rise inflammatory phenomena in the clinical picture of dermatosis it is accompanied by an increase in the number of eosinophils and granulocytes not only in the dermis, but also in the contents of the blisters; as they wither, mononuclear cells begin to predominate.

Clinical picture. Bullous pemphigoid is observed mainly in people over 60 years of age. Damage to the mucous membranes, unlike true pemphigus, is not inevitable, although it is not exceptionally rare. The disease, in good general condition, begins with the appearance of blisters on erythematous or erythematous-edematous spots, less often on apparently unchanged skin. Medium-sized blisters are 1-2 cm in diameter, hemispherical in shape, with a dense, tense covering, serous or serous-hemorrhagic contents. Due to the dense covering, they are more persistent than the blisters of true pemphigus. Erosions after their opening do not tend to grow peripherally and quickly epithelialize. When the contents of the blisters and erosion discharge dry out, yellowish and yellowish-brown crusts of various sizes and thicknesses are formed. The predominant localization is the lower half of the abdomen, inguinal folds, axillary fossae and flexor surfaces of the arms and legs. The lesions can be extensive, covering large areas of the skin, and very limited, represented by single blisters, for example, only in the navel area. Damage to the mucous membranes is observed in approximately 20-40% of patients with bullous pemphigoid and occurs, with rare exceptions, secondary; As a rule, it is limited to the oral cavity and occurs without severe pain and profuse salivation, the red border of the lips remains free from rashes. As the process progresses, and sometimes from the very beginning, its blisters spread across the skin until a generalized and even universal rash is formed. In rare cases, large blisters with a diameter of 5 to 10 cm are formed, followed by extensive erosive surfaces, or at the beginning of the disease, blisters, urticaria-like elements and papules appear, which are replaced by blisters over time. The rash of bullous pemphigoid is often accompanied by itching varying intensity, burning and soreness. The course of bullous pemphigoid is chronic, sometimes lasting for many years: it can be interrupted by remissions, usually incomplete. Relapses are often caused by UV rays, both natural and artificial. Over time, the severity of the disease gradually weakens and recovery occurs. However, such a successful outcome does not always occur: bullous pemphigoid is potentially serious illness, which does not exclude death.

The diagnosis of bullous pemphigoid is based on clinical and histological data and the results of indirect and direct immunofluorescence studies. It is especially difficult to differentiate pemphigoid from pemphigus vulgaris, especially in its initial stages, when acantholytic cells are often not detected. Final diagnosis help to establish the results of histological (subepidermal, not intraepidermal location of the bladder) and immunofluorescent (luminescence in the area of ​​the basement membrane, and not in the area of ​​the spinous layer) studies. The disease is often differentiated from Dühring's dermatitis herpetiformis.

Treatment: corticosteroid hormones are prescribed (40-80 mg prednisolone per day). The duration of treatment and the rate of reduction in the daily dose are determined by the severity of the disease. Cytostatics are also used (azathioprine, cyclophosphamide, methotrexate), as with true pemphigus. The detection of IgA antibodies in the basement membrane or a predominantly neutrophilic infiltrate serves as an indication for the prescription of sulfone drugs, in particular diaminodiphenyl sulfone, avlosulfone 50 mg twice a day according to the regimens adopted for dermatitis herpetiformis. External therapy similar to that performed for pemphigus. The prognosis is more favorable than with true pemphigus.

Dühring's dermatitis herpetiformis is a chronic recurrent skin disease characterized by a true polymorphism of the rash (vesicles, blisters, papules, blisters, erythema) and severe itching.

Etiology and pathogenesis. Etiology unknown. The autoimmune nature of the disease is assumed, as evidenced by gluten-sensitive enteropathy found in the vast majority of patients and the detection of IgA deposits (antibodies against the structural components of the dermal papillae near the basement membrane) in the dermoepidermal junction by direct immunofluorescence. IgA deposits are located predominantly in the form of granules at the apex of the dermal papillae and inside them. In some patients, circulating gluten-antibody immune complexes (IgA) are also detected. Play a certain role in the pathogenesis of the disease increased sensitivity to iodine and genetic predisposition. Dermatitis herpetiformis can also be observed as para-oncological dermatosis.

Histologically, subepidermal blisters are identified, the serous contents of which are often rich in eosinophils. The blisters are bordered by papillary microabscesses (accumulations of neutrophilic and eosinophilic granulocytes) located at the tops of the dermal papillae. Blood vessels the dermis is expanded and surrounded by infiltrates formed by neutrophils, eosinophils, destroyed nuclei (“nuclear dust”) and mononuclear cells with an admixture of neutrophilic leukocytes. Over time, the infiltrate becomes continuous, usually with a predominance of eosinophils. The bottom of subepidermal blisters can gradually become covered with regenerating epidermis.

Clinical picture. The disease occurs at any age, somewhat more often in 30-40 years. The provoking role of taking large amounts of starch and iodine, excessive insolation and viral diseases. The onset of the disease is usually gradual, lasting weeks and months. The disease becomes chronic and is interrupted by remissions lasting from 3 months to 1 year or more. Skin rashes may be preceded by malaise, mild fever, a tingling sensation and, especially often, itching. Characteristic is the true polymorphism of the rash, caused by a combination of erythematous spots, urticaria-like efflorescences, papules and vesicles, to which blisters can be attached. True polymorphism is complemented by false polymorphism (erosions, excoriations, crusts). Erythematous spots are usually small, round, have fairly clear boundaries; due to the addition of effusion from dilated vessels, they are transformed into urticaria-like formations, prone to peripheral growth and merging with each other into extensive foci of pink-bluish color, round, and often scalloped or bizarre outlines with clear boundaries. Their surface is dotted with excoriations, serous and hemorrhagic crusts, vesicles, usually located in the form of rings with a diameter of 2-3 cm or more. When infiltrate is deposited, erythematous spots transform into succulent pink-red papules with an initially smooth surface, which over time acquire pruriginous features. Urticariform and papular rashes can occur without a preliminary erythematous stage. Small vesicles (2-3 mm in diameter) appear on affected or apparently healthy skin, are distinguished by a dense covering and transparent contents, which become cloudy over time and can become purulent. When the contents of the vesicles dry out, crusts form, and when they are opened, which most often occurs under the influence of scratching, erosions are exposed. The vesicles, grouped together, resemble herpes rashes. Bubbles have the same clinical and evolutionary characteristics as vesicles, but differ from the latter only large sizes(their diameter is from 0.5 to 2 cm or more). The rashes are usually symmetrical; located on the extensor surfaces of the arms and legs, elbows, knees and shoulders, as well as on the sacrum, buttocks, lower back, back of the neck, scalp and face. They often group together. Damage to the mucous membranes is uncharacteristic; Only occasionally do vesiculobullous elements appear in the oral cavity, followed by their transition to erosion. With regression of rashes of dermatitis herpetiformis, as a rule, hypo- and hyperpigmented spots remain. Subjectively, severe itching, sometimes to the point of burning, and sometimes pain are noted. The general condition of patients during relapses may be disturbed: body temperature rises, itching intensifies, and sleep is disturbed. Laboratory changes include frequent eosinophilia in the blood and cystic fluid.

Diagnosis is based on clinical data. To confirm the diagnosis, determine the number of eosinophils in the blood and cystic fluid. An increased level in both of these fluids or in one of them indicates a diagnosis of dermatitis herpetiformis, however, the absence of eosinophilia does not exclude it; iodine test (Jadassohn test) is used in two modifications: cutaneously and orally. For 1 sq. cm of apparently healthy skin, preferably the forearm, apply ointment with 50% potassium iodide under a compress for 24 hours. The test is considered positive if erythema, vesicles or papules appear at the site of application of the ointment. If the result is negative, 2-3 tablespoons of a 3-5% potassium iodide solution are prescribed orally. The test is considered positive when signs of exacerbation of the disease appear. At severe course In case of dermatitis herpetiformis, an internal test can cause a sharp exacerbation of the disease, so it should not be performed in such cases. The most reliable results are histological examination, which allows one to detect a subepidermal blister, papillary microabscesses and “nuclear dust”. Particularly valuable are direct immunofluorescence data that reveal IgA deposits located in granules or linearly in the area of ​​the epidermal-dermal junction. Differential diagnosis is carried out with bullous pemphigoid, pemphigus, bullous toxiderma.

Treatment: Patients with dermatitis herpetiformis should be examined for the presence of concomitant diseases, primarily gastrointestinal, focal infection, oncological, especially with atypical forms of the disease in the streets of the elderly and old age. Diet is important: foods containing iodine and gluten are excluded from the diet. The most effective are sulfonic agents: diaphenylsulfone (DDS, dapsone, avlosulfone), diucifon, sulfapyridine, etc. Diaphenylsulfone or diucifon are usually prescribed 0.05-0.1 g 2 times a day in cycles of 5-6 days at intervals of 1-3 days . The course dose depends on the effectiveness and tolerability of the drug. If the clinical manifestations of dermatitis herpetiformis are resistant to sulfone drugs, corticosteroid hormones are indicated in average daily doses. Warm baths with potassium permanganate are prescribed locally; blisters and bubbles are opened, then treated with fucorcin or an aqueous solution of dyes; 5% dermatol ointment; corticosteroid ointments and aerosols.

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True pemphigus- a chronic autoimmune disease, which is characterized by the appearance of blisters on apparently unchanged skin and/or mucous membranes, located intraepidermally and formed as a result of acantholysis.

Epidemiology

Frequency of occurrence of true pemphigus among all skin diseases is 0.7-1%, and vulgar (ordinary) pemphigus accounts for up to 80% of cases of true pemphigus. Women get sick more often after 40 years, in last years cases of illness in young people aged 18 to 25 have become more frequent; Children and adolescents suffer from true pemphigus extremely rarely.

Classification

Distinguish 4 clinical varieties of true pemphigus:
■ vulgar (ordinary);
■ vegetative;
■ leaf-shaped;
■ erythematous (seborrheic).

Etiology

The etiology of the disease is unknown. 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, biological factors, in particular, the action of retroviruses is possible against the background of increased activity of proteolytic enzymes involved in the mechanisms of development of acantholysis.

Pathogenesis

As a result of the damaging effect on epidermal cells and the production of specific IgG, the connections between epidermal cells are destroyed, followed by the formation of blisters. The leading link in the morphogenesis of true pemphigus is acantholysis, which is caused by the interaction of pemphigus IgG with glycoproteins of cell membranes (pemphigus antigens), resulting in the destruction of desmosomes and disruption of the ability of keratinocytes to reproduce.

Risk factors for the development of true pemphigus may include various exogenous and endogenous factors(including genetic predisposition).

Clinical signs and symptoms of true pemphigus

All clinical forms 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: 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, nasopharynx, oropharynx, and genitals.
The first rash most often appears on the mucous membranes of the mouth, nose, pharynx and/or red border of the lips. For a long time patients are seen by dentists or ENT doctors for stomatitis, gingivitis, rhinitis, laryngitis, etc. Patients are bothered by pain when eating, talking, and when swallowing saliva. Characteristic sign- hypersalivation and specific odor from the mouth.
Then, after 3-6 months or within a year, the process becomes more widespread with damage to the skin. Blisters persist for a short time, and on the mucous membranes their appearance sometimes goes unnoticed, due to the fact that the covers of the blisters are thin and 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. The peculiarity of erosions is a tendency to peripheral growth, while 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 outcome in the absence of treatment. Nikolsky's symptom (separation of the layers of the epidermis overlying the basal layer, with minor mechanical impact) can be positive both in the lesion and near it, and even on apparently healthy skin far from the lesion.

Seborrheic, or erythematous, pemphigus (Senir-Usher syndrome): unlike pemphigus vulgaris, which first affects the mucous membranes, the process begins in seborrheic areas (on the skin of the face, back, chest, scalp).
At the beginning of the disease, erythematous lesions with clear boundaries appear on the face, 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 grayish-yellowish crusts, which when peeled away reveal an eroded surface. The blisters have a very thin, flabby covering that lasts for a short time, so the blisters often go unnoticed by patients and doctors. Nikolsky's sign is positive in the lesions. Due to the presence of fairly clear boundaries of the lesion, crusts or scale-crusts, erythema, exacerbation of the process during insolation, seborrheic pemphigus often has to be differentiated from seborrheic dermatitis or discoid lupus erythematosus. The disease may be limited for months or years, then it may spread to affect new areas of the skin and mucous membranes (usually the oral cavity); when the process generalizes, the disease acquires the features of pemphigus vulgaris.

Pemphigus foliaceus characterized by erythemato-squamous rashes, thin-walled blisters, repeatedly appearing 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 not typical. 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. Death may occur from sepsis or cachexia. Nikolsky's symptom is positive even on apparently healthy skin.

Pemphigus vegetans can proceed benignly for many years, having limited lesions with satisfactory condition sick. Bubbles most often appear on the mucous membranes of the oral cavity, around natural openings (mouth, nose, genitals, etc.) and in the area of ​​skin folds (axillary, inguinal, behind-the-ear, under the mammary glands). At the bottom of the erosions, soft, juicy, fetid vegetations are formed, covered with serous and/or purulent plaque with the presence of pustules along the periphery, which makes it possible to differentiate this disease from chronic vegetative pyoderma. Nikolsky's symptom is positive only near the lesions. In the terminal stage, the skin process is very similar to pemphigus vulgaris.
The division of true pemphigus into various clinical forms is arbitrary, because the clinical picture of one type may resemble that of another, and a transition from one form to another is also possible. The principles of therapy do not differ significantly for different clinical forms ah, at the same time, there are some features of patient management, which will be described below.

The diagnosis of true pemphigus is based on clinical picture disease, positive Nikolsky’s symptom and its modifications (the “pear” phenomenon described by N.D. Sheklakov, G. Asboe-Hansen’s symptom), which are based on the phenomenon of acantholysis, and laboratory methods research such as:
■ cytological analysis for the presence of acantholytic cells in impression smears from the bottom of erosions (the presence of acantholytic cells in blisters is not pathognomonic, but a very important diagnostic sign of the disease);
■ histological examination (allows us to detect the intraepidermal location of cracks and blisters);
■ direct immunofluorescence method (determines the presence of class G immunoglobulins in the intercellular adhesive substance of the epidermis).

Differential diagnosis

Carry 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 vegetative pyoderma.

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

It is characterized, in contrast to true pemphigus, by a multimorphic itchy rash, dense tense blisters on an edematous hyperemic base, rapid epithelization of erosions, the absence of Nikolsky's sign and acantholytic cells in the smear-imprint from the bottom of erosions, subepidermal location of blisters, deposition of immunoglobulin A in the area of ​​the dermal papillae, high content eosinophils in cystic fluid and/or peripheral blood.

For chronic benign familial pemphigus Gougereau-Hailey-Hailey hallmark is the family nature of the lesion, a benign course, worsening in the summer, the presence of lesions in favorite places (the side of the neck, axillary, inguinal folds, the navel area), the presence of maceration with the formation of tortuous fissures like cerebral gyri, pathognomonic for this disease. Nikolsky's symptom is not always positive and only in lesions. Acantholytic cells are found, but without signs of degeneration; Deposition of immunoglobulins is not typical. The disease occurs with periods of remissions and exacerbations; it does not require permanent treatment. The rashes often respond to regression even when only local therapy is prescribed without the use of systemic drugs.

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 absent. Nikolsky's symptom is negative.

Seborrheic dermatitis, despite the similarity with seborrheic pemphigus, it is quite easy to distinguish from it due to the absence of clinical and laboratory signs of acantholysis, damage to the mucous membranes, histological and immunofluorescent signs characteristic of true pemphigus.
Unlike true pemphigus Lyell's syndrome- an acute severe illness that is accompanied by fever, polymorphism of rashes and is usually associated with taking medications.
For chronic vegetative pyoderma In addition to signs reminiscent of pemphigus vegetans, symptoms of severe pyoderma are characteristic: erosions, ulcers, deep folliculitis. At the same time, Nikolsky’s symptom is negative, and there are no laboratory signs of true pemphigus.

Determination of Nikolsky's symptom has exceptional diagnostic value for true (acantholytic) pemphigus and epidermolysis. When we pull on a piece of the bladder cover, we observe detachment of the upper layers of the epidermis within the boundaries of healthy skin outside the base of the bladder. This phenomenon is caused by acantholysis or epidermolysis. Nikolsky's symptom can also occur with familial pemphigus, bullous pemphigoid, acute febrile pemphigus, and Lyell's toxic necrolysis. Lightly rubbing the skin between the two blisters with a finger will also cause the epidermis to peel off. In some cases, the symptom may be positive in areas distant from the blisters (pemphigus foliaceus).

When pressing with a finger on an intact bladder, its area increases, the tire becomes flabby, since fluid pressure leads to detachment of the epidermis in the opposite direction from the place of pressure (Asbo-Hansen's symptom - a variant of Nikolsky's symptom). When the patient remains in one position for a long time (sitting, lying down), a “spontaneous” Nikolsky symptom (Sheklakov’s “pear” symptom) may be detected if the liquid constantly presses on the edge of the bladder, exfoliating the epidermis.

Nikolsky's symptom helps to differentiate acantholytic pemphigus from the bullous form of exudative erythema multiforme, non-acantholytic pemphigus, Galoppo's pyoderma vegetans, Dühring's and Darier's diseases, and subcorneal pustulosis.

4. Setting up the Jadassohn test

The Jadasson test is performed to clarify the diagnosis of Dühring's dermatosis herpetiformis. If necessary, three stages are performed sequentially if the result of the first of them is doubtful.

First stage. Apply 50% potassium iodide ointment under a compress to an area of ​​1 cm2 of skin that is free from rashes. To control the symmetrical area, apply a compress with Vaseline. At positive test 24 hours after applying K1, rashes appear, which are accompanied by itching.

Second phase. A compress with potassium iodide is applied to the area of ​​skin where the rash already exists. If the test is positive, old rashes become brighter and the itching intensifies significantly.

Third stage. During the day, the patient is given 1 tablespoon of a 3% aqueous solution of potassium iodide to drink 2-3 times. If the test is positive, fresh rashes appear on any area of ​​the skin, which are accompanied by intense itching.

5. Examination of fingerprint smears for acantholytic cells

For the first time, the cytological method for diagnosing blistering dermatoses was proposed by A. Tzank. This method is indispensable in the differential diagnosis of acantholytic pemphigus, pemphigoid and Dühring's dermatosis herpetiformis.

Using a piece of boiling-sterilized gum, lightly press the surface of the bottom of the fresh bubble. The material is transferred to a fat-free sterile glass slide, fixed with methanol for 1 minute, dried at room temperature and stained according to Romanovsky - Giemsa (20-25 minutes with azureosine). The drug is examined under a microscope at a magnification of 10x40.

Acantholytic cells are degenerative-dystrophic. They are smaller than normal epithelial cells, have a round shape, a large nucleus that occupies almost the entire cell and are intensely stained. Two or more lighter nucleoli are visible in the nucleus. The cytoplasm is visible along the periphery in the form of a rim. She is sharply basophilic.

Etiopathogenesis. Mostly women aged 40-60 years are affected, but it is possible that people of any age group may be affected. The etiopathogenesis is not fully understood. An important role in the development of the disease is played by dysfunction of the adrenal cortex, disturbances of water and especially salt metabolism, as evidenced by a sharp decrease in the daily excretion of sodium chloride in the urine, degenerative changes in the brain, spinal cord and intervertebral ganglia, and disturbances of enzyme systems. A promising theory is that pemphigus is caused by a member of a group of retroviruses in the presence of a genetic predisposition.

The pathogenesis of pemphigus is based on autoimmune processes, the essence of which is the formation of autoantibodies to the cementing intercellular substance (desmosomes) and membranes of cells of the spinous layer of the epidermis under the influence of changes in their antigenic structure, presumably induced in turn by altered nuclear DNA. They are called "pemphigus-like" antibodies; by their nature they belong to IgG and are detected in the direct immunofluorescence reaction in the form of fixed antigen-antibody complexes at the sites of bubble formation; can be detected in the epidermis of apparently unaffected skin, as well as in the blood serum of patients with pemphigus. They determine the formation of the pathohistological process - acantholysis - the leading link in the pathogenesis of pemphigus; under their influence, the intercellular substance dissolves, desmosomes are destroyed and the ability of epidermal cells to reproduce is lost. According to this concept, acantholysis in pemphigus is primary, and cell damage is secondary.

However, this theory cannot be accepted unconditionally. In 10–25% of patients with pemphigus, neither fixed nor circulating “pemphigus-like” antibodies can be detected. At the same time, similar antibodies can be detected in blood serum in cases of extensive burns, Lyell's syndrome, penicillin toxiderma, bullous pemphigoid, systemic lupus erythematosus and other pathological processes. These circumstances cast doubt on the idea that “pemphigus-like” antibodies cause acantholysis, despite their fixation in the sites of development of blisters in the vast majority of patients with pemphigus.

N.S. Potekaev et al. a new concept of the pathogenesis of pemphigus is substantiated, the basis of which is the syndrome of regenerative-plastic deficiency caused by defective synthesis of protein structures of unaffected keratinocytes, which leads to their functional and morphological damage. This pathological process initially occurs in the nucleus of cells in the basal layer. In spiny cells, the increasing changes in the nucleus are accompanied by a change cytoplasmic organelles, primarily functionally leading - tonofibrils and associated desmosomes, up to their dissolution, which leads to the formation of acantholysis. The primary cause of these damages is a decrease in the synthesis of DNA-dependent RNA in keratinocytes under the influence of some influence (retrovirus?) on the genetic apparatus of the cell. This complex pathological process is accompanied by a change in the antigenic composition of epidermal cells, which leads to the production of “pemphigus-like” antibodies. Thus, according to this point of view, acantholysis and the formation of “pemphigus-like” antibodies are the final stage of morphogenesis in pemphigus, and not its beginning.

Autoimmune disorders in pemphigus are undoubtedly important in the applied aspect: the detection of “pemphigus-like” antibodies, especially fixed ones, indicates the presence of this disease, and based on the titer of circulating antibodies and its dynamics, one can predict the course of the disease, evaluate the results of treatment and make adjustments to it.

Acantholysis underlies important differential diagnostic signs of pemphigus - Nikolsky, Asbo-Hansen, “pear” symptoms, as well as the identification of acantholytic cells with a smear-imprint with a sterile student eraser from the bottom of the blisters (Tzanck cells).

Acantholytic cells are modified spinous cells of the epidermis. Their features:

1) the size is smaller than normal spinous cells of the epidermis, but the nucleus is several times larger;

2) cell nuclei are intensely stained;

3) cells often contain several nuclei;

4) 2-3 large nucleoli are found in the enlarged nucleus;

5) the cytoplasm is basophilic and unevenly stained: around the nucleus there is a light blue zone, and along the periphery there is an intense blue rim (“concentration rim”);

Tzanck cells are not a pathognomonic feature of true pemphigus. Similar cells can be found in herpes simplex and herpes zoster, chicken pox, chronic benign familial pemphigus, pustular psoriasis and a number of other dermatoses. However, the cytological method facilitates the recognition of pemphigus.

Pemphigus is a monomorphic dermatosis with intraepidermal blisters.

There are several clinical forms of true pemphigus: vulgar, vegetative, foliate, seborrheic and Brazilian.

Pemphigus vulgaris

Most common.

The disease begins with damage to the oral mucosa, namely the pharynx, which is facilitated by sore throat, flu, tooth extraction and prosthetics. The blisters are initially single, have a thin, flabby covering, which quickly opens with the formation of painful bright red erosions, along the periphery of which individual fragments of whitish epithelium are visible. Eating is difficult or almost impossible due to severe pain; erosions on the red border of the lips are covered with hemorrhagic and serous crusts. When the larynx and pharynx are affected, the voice becomes hoarse. There is a heavy putrid odor from the mouth. The general condition of the patients remains satisfactory; regressed rashes are replaced by new ones.

The localized process lasts from several days to 3-6 months or more, then the skin is involved in the process and generalization of the process occurs, which is characterized by the rapid spread of rashes throughout the entire skin. The blisters can reach large sizes - up to the size of a chicken egg or more, they can merge with each other, their tire is flabby, and the contents are cloudy or purulent. Extensive erosions are bordered by fragments of the epidermis, have a bright red or bluish-pink color, and are covered with serous exudate.

With the generalization of skin rashes, there is a deterioration in the general condition of the patient: weakness, malaise, loss of appetite, insomnia, fever up to 38-39 degrees, diarrhea, swelling; joins secondary infection, cachexia, intoxication, and dehydration develop. Without treatment - death. Positive symptoms of Nikolsky (marginal and distant), Asbo-Hansen. Subjectively – pain, burning, itching.

Pemphigus vegetans

It is characterized by a predominance of vegetative elements, i.e., papillomatous growths and a more benign course. Bubbles are also initially located on the oral mucosa, then mainly around natural openings and in skin folds. When the blisters are opened, bright red weeping erosions are exposed, on their surface succulent vegetation of pink-red color, soft consistency, 0.1 - 1 cm or more in height is formed; their surface is covered with a grayish coating, serous or purulent discharge, and crusts. There is a foul odor.

Subjectively – pain and itching of varying intensity. The course of pemphigus vegetans is longer than that of pemphigus vulgaris; there may be long-term remissions (several months or years). Nikolsky's sign is positive near the lesions.

Pemphigus foliaceus (exfoliative)

It is less common vulgar, but more often vegetative. The mucous membranes, with rare exceptions, are not affected.

Characteristic are small, flat blisters with a thin and flabby covering that appear on an erythematous background. Superficial erosions are pink-red in color, superficial with copious serous discharge, which dries into lamellar crusts. Thin lamellar crusts resemble sheets of paper, hence the name of this dermatosis. Usually the crusts are not rejected, but are layered on top of each other and massive layered formations are formed.

In addition to bubbles as primary elements There may be scaly papules and pink-red spots.

There is often a rapid spread of the process throughout the entire skin with the appearance of exudative erythroderma: the affected skin is diffusely hyperemic, swollen and infiltrated, covered with flabby blisters, weeping erosions, hyperkeratotic scales and layered crusts. Often hair falls out and nails are torn off.

Nikolsky's symptom is sharply positive. Subjectively – itching, because in the blood – eosinophilia up to 40% or more, pain. The course is long - 2-5 years or more.

Pemphigus erythematous (seborrheic, Senir-Usher syndrome)

It usually begins with damage to the face or scalp, followed by spread to the chest, interscapular area, large folds and other areas of the skin. The mucous membranes and conjunctiva of the eyes are rarely affected.

Initially, pink-red plaques with a diameter of 2-5 cm with clear boundaries, rounded or irregular outlines appear. Their surface can be covered either with dry scales, on back side soft spines, or greasy yellowish-brown crusts, after the removal of which weeping erosions are exposed. Tzanck cells “+” are identified. The central part of the plaques is sunken, and the peripheral part is bordered by a fringe of exfoliating epidermis.

Nikolsky's symptom is positive, marginal. Over time, after 2-3 weeks, or 2-3 years or more, blisters appear, as with pemphigus vulgaris or pemphigus foliaceus, which can lead to the transformation of seborrheic pemphigus into these forms. Exacerbation and transformation of pemphigus are provoked by insolation.

Pemphigus Brazilian (tropical) is a variant of pemphigus foliaceus found in southwestern Brazil and surrounding countries.

Differential diagnosis of true pemphigus is carried out with: Dühring's dermatitis herpetiformis, Lever's bullous pemphigoid, Lyell's syndrome, seborrheic eczema.

Diet, rich in vitamins and proteins, with a sharp limitation of carbohydrates, fats and table salt.

1. Basics medicine– corticosteroid hormones. Prednisolone is still the drug of choice. The initial dose is 120-180 mg/day or more. Extremely sick in serious condition Pulse therapy is indicated - 1000 mg of prednisolone per day intravenously once. With the correct dose, the therapeutic effect (no fresh blisters, erosions heal well) occurs on days 10-14, then a gradual (to avoid withdrawal syndrome) reduction of the hormone begins by 2.5 - 5.0 mg every 3-5 days.

When the daily dose reaches 20-30 mg, further reduction in order to avoid relapse of the disease should be carried out with great caution to a maintenance dose of 5.0-2.5 mg.

In addition to prednisolone, triamcinolone, medipred, dexamethasone and others are used hormonal drugs. The number of their tablets is equivalent to the number of prednisolone tablets.

It is necessary to remember the complications of steroid therapy: the development of Itsenko-Cushing syndrome, obesity, steroid diabetes, erosive and ulcerative pathology of the gastrointestinal tract, hypertension, insomnia, acute psychosis, myocardial infarction. Systemic angiopathy occupies a special place among the complications of hormone therapy. Her cutaneous manifestation– “corticosteroid” skin: subatrophy, dry skin, a pronounced tendency to the appearance of hemorrhagic spots on it in response to mechanical irritation.

In patients with true pemphigus, to avoid side effects hormone therapy every 10 days must be examined general analysis blood test, general urine test, blood sugar test, stool test for occult blood, daily blood pressure monitoring, if necessary, consultation with a therapist and related specialists.

2. Potassium salts (up to 3 g per day): panangin, asparkam, potassium orotate. They are necessary to maintain the functioning of the heart muscle and restore water and electrolyte balance. Products include baked potatoes.

3. Calcium salts in the form of calcium chloride intravenously, calcium gluconate intramuscularly, calcium pantothenate orally to prevent osteoporosis.

4. Antibiotic therapy to prevent secondary infection.

5. Antihistamines, hyposensitizing drugs.

6. Vitamin therapy.

7. Hemodesis and other blood substitutes to normalize water and electrolyte balance and relieve dehydration.

8. Cytostatic agents are prescribed as agents that complement corticosteroid therapy, especially in severe cases of pemphigus: methotrexate, azathioprine, etc. The duration of their use depends on the therapeutic effect. Methotrexate – administered intramuscularly at 25 mg once a week; for a course of 6-8 injections. The number of courses and the intervals between them are determined by the severity of the skin process or in tablets according to Vanstein: 25 mg once a week in 3 doses.

Complications during treatment with cytostatics: ulceration of the gastrointestinal mucosa, dysfunction of the liver, kidneys and pancreas, hematopoiesis, alopecia. Indications for their cancellation: leukopenia below 4.109, a decrease in hematocrit by 10% or more, diarrhea, fresh gastrointestinal ulcers.

9. Anabolic steroids (retabolil, nerabol).

10. Local treatment – aqueous solutions aniline dyes, corticosteroid ointments, irrigation with anesthetics, polocortolone. The prognosis for pemphigus is always serious; patients should be under medical supervision.