True bladder. The reasons. Symptoms. Diagnostics. Treatment. Pemphigus disease - how to cope with the disease

Pemphigus is a serious disease that affects the human skin. As a result of its progression, pathological blisters are formed on the skin and mucous membranes, filled with exudate inside. This process begins due to the stratification of the epithelium. Pathological foci can merge and tend to grow rapidly.

To date, the true causes that provoke the progression of the disease have not yet been precisely established. Therefore, treatment in most cases is ineffective.

Pemphigus of newborns is especially dangerous, as it develops rapidly, and without timely and adequate treatment can lead to the development of serious complications or even death. But it is worth noting that children suffer from this disease is extremely rare. Much more often, viral pemphigus of the mouth and extremities, as well as other parts of the body, is diagnosed in people aged 40 to 60 years.

The reasons

Clinicians cannot name the exact etiology of viral pemphigus. But now there is an assumption about the autoimmune origin of the disease.

Also, scientists recently found that the cause of the progression of pemphigus in newborns is the penetration of bacteria into the body, the most dangerous of which is golden. The source of infectious agents can be a person who is in the close environment of the child. The causative agent can be easily transmitted to a newborn through close contact.

Classification

In medicine, several classifications of the disease are used, which are based on the manifestation of the pathological process, its varieties, the characteristics of the course of the disease, as well as the severity.

Basic forms:

true or acantholytic pemphigus. It combines several types of diseases that are the most dangerous for humans, and can provoke the progression of complications that are dangerous not only for health, but also for human life;
benign or nonacantholytic pemphigus. It is a less dangerous pathology. It flows relatively easily.

Depending on the characteristics of the course of the disease and the severity:

mild degree. Pathological elements on the skin are formed gradually. The number of foci is minimal;
moderate. This form is characterized by the formation of blisters on the skin and oral mucosa. The number of foci increases;
heavy. Most of the skin is affected. Ulcers can be found in the mouth. The foci of inflammation merge and form large conglomerates. Associated pathologies develop.

Varieties of true pemphigus

Pemphigus ordinary. This type of pathology is diagnosed in patients more often. Blisters form on the skin, but there are no signs of inflammation. If the treatment of pemphigus is not carried out on time, then pathological elements can spread throughout the skin. It is worth noting that they can merge and form large lesions.

erythematous form. This pathological process combines several diseases. Its symptoms are similar to the erythematous variant of the systemic, as well as true pemphigus. Erythematous pemphigus in adults and children is very difficult to treat. It is worth noting that the disease is diagnosed not only in humans, but also in some animals. A characteristic symptom is the appearance on the skin of the body and face of red spots, covered with crusts on top. Simultaneously with this symptom, seborrheic manifestations appear on the scalp.

Brazilian bladderwort. Has no restrictions regarding gender and age. Cases of its development have been recorded in young children and in older people aged 70 to 80 years. Also, its progression in middle-aged people is not excluded. It is worth noting that this species is endemic, therefore it is found only in Brazil (hence the name).

Leaf-shaped bladder. The name of this form was due to the characteristics of pathological elements. Bubbles form on the human skin, which practically do not rise above the epidermis (not tense). On top of them, crusts are formed that have the property of layering on top of each other. Creates the effect of sheet material stacked.

Classification of nonacantholytic pemphigus

Bullous form of nonacantholytic pemphigus. This is a benign disease that develops in both adults and children. Blisters form on the skin, but there are no signs of acantholysis. These pathological elements can spontaneously disappear without scarring.

Neacantholytic pemphigus benign character. Pathological elements are formed exclusively in the human oral cavity. On examination, inflammation of the mucosa, as well as its slight ulceration, can be detected.

Scarring nonacantholytic pemphigus. This pemphigoid is referred to in the medical literature as pemphigus of the eye. Most often, it is diagnosed in women who have crossed the 45-year age limit. A characteristic symptom is damage to the visual apparatus, skin and oral mucosa.

Symptoms

Viral pemphigus in children and adults is a disease that has similar symptoms, regardless of its type. A feature of acantholytic and non-acantholytic pemphigus is an undulating course. If timely assistance is not provided, the patient's condition will rapidly deteriorate.

General symptoms:

loss of appetite;
weakness;
symptoms of cachexia increase;
erosion epithelialization slows down.

Symptoms of the disease, depending on which form began to progress in a person.

With pemphigus vulgaris, blisters of various sizes form on the skin. They have a sluggish and thin-walled shell. First they are localized in the oral cavity. Usually, a sick person, after discovering pathological elements, goes to see a dentist, but in fact, a dermatologist is also involved in the diagnosis and treatment of pemphigus vulgaris. The main complaints: pain when talking and eating, an unpleasant smell from the oral cavity.

Symptoms of the disease persist for 3-12 months. If during this period you do not take up treatment, then the pathological formations will begin to spread and pass to the skin. In severe cases, intoxication syndrome and the addition of a secondary infection are observed.

The size of the blisters in the erythematous form is relatively small. The walls of the bladder are thin and sluggish. Formations can spontaneously open. It is very difficult to diagnose such pemphigus in children and adults.

The leaf form affects mainly the skin, but sometimes spreads to the mucous membrane. Its main difference from other forms is the simultaneous formation of pathological blisters and crusts that overlap each other. With such a disease, development is possible, which often leads to the death of the patient.

Neacantholytic pemphigus often begins to progress in the elderly. Its course is chronic. Bubbles form on the mucous membrane and on the lips. They can open, after which scars do not form, and atrophic areas do not appear.

Bullous dermatitis - blisters form on the mucosa, do not have a border from the exfoliating epithelium on the periphery. Not painful when touched. Against the background of the disease, there is a lesion of the esophagus, chronic rhinitis, and so on.

Diagnostics

In order to accurately establish the presence of viral pemphigus in children and adults or pemphigus in newborns, it is necessary to conduct a comprehensive examination of the patient, which includes several stages:

inspection and clarification of symptoms;
Nikolsky's test - a technique that allows you to accurately differentiate pemphigus from other pathological processes that have a clinic similar to it;
cytological analysis;
histological examination;
direct immunofluorescence method.

Treatment

To date, several methods are used to treat the disease:

drug therapy;
local treatment;
extracorporeal methods.

Drug therapy includes the appointment of the following groups of drugs:

antibiotics;
corticosteroids;
cytostatics.

Local treatment:

ointments containing corticosteroids;
baths with the addition of potassium permanganate;
irrigation of the surface of the mucous membrane and skin with anesthetic solutions;
use of aniline dyes.

Extracorporeal techniques:

hemosorption;
hemodialysis;
plasmapheresis.

Is everything correct in the article from a medical point of view?

Answer only if you have proven medical knowledge

True pemphigus (pemphigus) (synonym: acantholytic pemphigus) is a severe chronically relapsing, autoimmune disease of the skin and mucous membranes, the morphological basis of which is the process of acantholysis - a violation of the bonds between the cells of the epidermis. Blisters are caused by acantholysis. The mechanism of immune disorders has not been fully established.

People of all nationalities suffer from pemphigus, but people of Jewish nationality suffer more often. The disease is also often recorded in Mediterranean peoples (Greeks, Arabs, Italians, etc.), in eastern India. Such a frequent occurrence of pemphigus may be due to consanguineous marriages allowed in some nationalities. Most of the scientific literature on the problem of pemphigus indicates the predominance of this dermatosis among women.

Causes and pathogenesis of true pemphigus

Despite numerous studies, the etiology and pathogenesis of pemphigus remain unknown. There are many theories explaining the occurrence of the disease: the theory of chloride retention, the theory of toxic origin, the theory of cytological anomalies, the theory of neurogenic origin, the endocrine theory, the theory of enzyme origin, the theory of autoimmune origin, etc. However, many existing theories are outdated and have only historical significance.

Based on currently available immunological data, pemphigus is an autoimmune disease, although the causes of impaired functioning of the immune system in this pathology remain unclear. Perhaps the change in the immune system occurs under the influence of exogenous factors in the presence of genetic features of the immune system.

Data on studies of cellular immunity are heterogeneous and they show an increase in IgG, serum autoantibodies, a decrease in T-cell immunity, a decrease in the proliferative response to T-cell mitogens, such as concanavalin A and phytohemagglutenin. However, the data obtained take place only in patients with a severe and widespread process.

There is a difference in the production of various IgG in patients in the acute phase of the disease and in remission. IgGl and IgG4 predominate in patients with exacerbations. It was revealed that IgG pemphigus antibodies fix both early (Clq, C3 C4) and late (C3-C9) complement components. Early complements can pre-accumulate in the keratinocyte membrane, which leads to the activation of later complements under the influence of pemphigus IgG. In this case, a complex is formed that disrupts the permeability of keratinocyte membranes.

Autoantibodies in the blood serum of patients with pemphigus are directed to antigens of the intercellular adhesive substance (desmosomes) of the stratified squamous epithelium, which correlates with disease activity.

Currently, three representatives of the desmogleins (Dcr) class are known. These are desmoglein-1 (Dcr1), desmoglein-2 (Dcr2), and desmoglein-3 (Dcr3). Their weight is encoded by genes located on the 18th chromosome, which confirms their relationship. Electron microscopic studies showed the localization of both Dcr1 and Dcr3 in desmosomes. Both desmogleins are well represented in stratified squamous epithelium and are associated with disulfide bonds with placoglobulins, proteins of desmosomal plaques. Dcr2 is the most abundant desmosome protein and is localized in the desmosomes of non-epithelial cells.

Immunohistochemical studies revealed that Dcr1 is an antigen for leaf-shaped, and Dcr3 - for pemphigus vulgaris. This approach to the pathogenesis of pemphigus allows us to categorically assert the presence of only two of its forms: vulgar and leaf-shaped (superficial). All other forms are their variants.

Symptoms of true pemphigus

Clinically, acantholytic pemphigus is divided into ordinary, vegetative, leaf-shaped and erythematous (seborrheic, or Senier-Uscher syndrome).

Pemphigus vulgaris is characterized by rashes of flaccid blisters, located, as a rule, on unchanged skin and mucous membranes, with transparent contents. The blisters quickly open with the formation of painful erosions that have a red weeping surface, increasing even with a slight injury. With friction, erosion can also occur on outwardly unchanged skin, especially near blisters (Nikolsky's symptom). Rashes can be located on any part of the skin, but the mucous membranes, skin folds, and areas that are traumatized are most often affected. In about 60% of patients, the process begins in the oral cavity and for a long time can be limited only to this area, resembling stomatitis. Isolated lesions, sometimes with vegetations, hyperkeratosis, especially in children, often having a significant resemblance to impetigo, seborrheic dermatitis, pink lichen, bullous exudative erythema multiforme and other dermatoses, can also be on the skin. For the purpose of diagnosis in such cases, smears-imprints from erosive surfaces are examined to detect acantholytic cells and an immunomorphological study is carried out to identify immune complexes in the pilermis.

Mixed forms with signs of pemphigus and pemphigoid are described, as well as variants resembling Dühring's herpetiform dermatitis. The disease develops in middle and old age, although it can also be observed in children.

Vulgar (ordinary) pemphigus usually has an acute character and in most patients (more than 60% of cases) begins with lesions of the oral mucosa, being the only symptom of the disease for a long time. Perhaps the onset of the disease with damage to the mucous membrane of the genital organs, larynx, trachea. Initially, single or few blisters appear, often located in the retromalar region, on the lateral surface of the tongue. Under the influence of food or teeth, the thin and flabby cover of the bubbles quickly opens and bright red erosions are exposed, along the periphery of which fragments of the covers of the bubbles can sometimes be observed. The eroded areas of the oral mucosa are very painful: patients cannot chew and swallow food, there is pronounced salivation, deep cracks in the corners of the mouth, which do not allow it to be opened. Patients with lesions of the oral mucosa sometimes go to the dentist and receive treatment for stomatitis for a long time. After 3-6 months, the appearance of single blisters on the skin is noted, and with progression, the generalization of the process begins. Pemphigus is characterized by the appearance of flaccid blisters (monomorphic nature of the rash) on apparently unchanged, rarely on erythromatous skin. Bubbles can be small or large, with serous, and after some time - cloudy, sometimes hemorrhagic contents. Over time, the bubbles tend to grow peripherally, merge with each other with the formation of large scalloped foci. After some time, the content of the blisters dries up, forming yellowish crusts, which, when they fall off, leave hyperpigmented secondary spots. If the lid of the bladder is damaged, bright red painful erosions with a juicy red bottom are formed, separating a thick exudate, along the periphery of which there are fragments of the lid of the bladder. During this period, Nikolsky's symptom is almost always positive (not only in the immediate vicinity of the lesion, but also in areas of apparently unchanged skin). The essence of this phenomenon is the detachment of the clinically unchanged epidermis with sliding pressure on its surface. A modification of Nikolsky's symptom is the Asbo-Hansen phenomenon: when pressing a finger on the tire of an unopened bladder, its area increases due to acantholysis.

The “pear” phenomenon was described by N. D. Sheklakov (1961): under the weight of the fluid accumulated in the bubble, with pronounced acantholysis phenomena, its base area increases and the bubble acquires a pear-shaped shape. The movement of patients due to the pain of erosion is limited.

Blisters may appear on an edematous and erythematous background and tend to cluster. It is believed that the blisters in pemphigus vulgaris occur on intact skin and the clinical picture is similar to Dühring's herpetiform dermatitis. In such cases, we are talking about herpetiform pemphigus. The following clinical, histological and immunomorphological criteria for pemphigus herpetiformis as a variant of pemphigus vulgaris are given in the literature:

herpetiform nature of rashes, accompanied by burning and itching;
suprabasal and subcorneal acantholysis with the formation of intraepidermal blisters;
detection of IgG in the intercellular space of the epidermis.

In the future, along with herpetiform rashes, patients increasingly develop large flaccid blisters on apparently unchanged skin, and the clinical picture takes on the classic features characteristic of pemphigus vulgaris.

Erosions slowly epithelialize and after healing of the foci on the mucous membranes and conjunctiva, no scars remain. In the case of a secondary infection or when the basement membrane is involved in the pathological process, areas of cicatricial atrophy or scars form at the site of the former lesions. The generalization of the process is often accompanied by a deterioration in the general condition of patients, malaise, weakness, insomnia, fever, and sometimes fever are noted. If treatment is not carried out, patients die from the addition of a secondary infection or cachexia.

Histopathology. In the lesions, there is a loss of intercellular bridges, in the deep layers of the epidermis - acanthosis, the formation of intraepidermal cavities. The blisters contain round acantholytic Tzank cells. In the intercellular spaces of the epidermis, antibodies of the IgG class are detected.

Histogenesis. Acantholysis is based on changes in the cementing substance that is in direct contact with the outer layer of the plasma membrane of epithelial cells and, to a greater extent, in desmosomes. It has been established that immune disorders play an important role in the primary damage to the cementing substance. By direct immunofluorescence, antibodies of the IgG type were detected in the skin. localized in the intercellular spaces of the epidermis. Indirect immunofluorescence revealed antibodies against the components of the intercellular cementing substance of the epidermis when treated with luminescent anti-IgG human serum. The C3 component of the complement was also found, which makes it possible to classify this disease as an immunocomplex disease.

The mechanisms of immune disorders in pemphigus have not yet been established. It is believed that the main pathogenetic role belongs to the antigen to desmoglein III circulating in the blood, which is a glycoprotein in combination with placoglobin and is a mediator of cell adhesion in the desmosome region. It is assumed that the beginning of the antigen-antibody reaction, which promotes acantholysis, is preceded by the activation of proteases and plasminogen activator. By immunochemical analysis of epidermal antigens E.P. Matushevskaya (1996) identified a previously unstudied antigen - water-soluble skin globulin a2-BGK. In addition, two specific proteins α2-HLPL-130 and α2-GPLP-160 associated were found in the cystic fluid. respectively, with ordinary and leaf-shaped forms of pemphigus. It points to damage to the immune system at various levels, including the thymus and skin, the possible role of genetic factors, suggested on the basis of family cases of the disease, data on the increased detection of certain tissue compatibility antigens. In particular, the association of the disease with HLA-A10, HLA-A26, HLA-DRW6, HLA-DRW4, BW38 has been established. It is believed that carriers of the DRw6 serotype have a 2.5-fold increased risk of developing the disease, and the predisposition to pemphigus disease is associated with linkage disequilibrium with the DQw3 and DQwl alleles of the DQ locus. A new allele (PV6beta) of the same locus was discovered and a test with PV6beta, an allele specific oligonucleotide, was proposed for diagnosing the disease at early stages or in atypical cases. The role of viral infection has not been proven. Basically, B-cell immunity changes, but with a long course, a defect in T-cells also develops. Insufficiency of interleukin-2 synthesis was revealed. Vegetative pemphigus is distinguished by the presence of papillomatous-verrucous growths in the area of erosion, localized mainly in the folds of the skin and periorifically. In some patients, lesions may be similar to those in vegetative pyoderma due to the appearance of vegetation from pustular elements (alopo vegetative pemphigus). The differential diagnostic sign in such cases is the detection by direct immunofluorescence of IgG, which form immune complexes with the antigen in the epidermis. Vegetative pemphigus of this type proceeds more favorably than the classic version of Neumann.

Pathomorphology. Acanthosis with elongation of epidermal outgrowths and papillae of the dermis and proliferation of cells of epithelial cords. In the area of verrucous vegetation - acanthosis, papillomatosis, intraepidermal abscesses containing eosinophilic granulocytes. The presence of these abscesses is characteristic of vegetative pemphigus. With the Allopo type, in the foci, which are blisters-pustules, acantholysis is observed with the formation of small suprabasal slits around the pustules. The cavities are filled with eosinophilic granulocytes and acantholytic cells.

To ensure a correct diagnosis, it is necessary to biopsy the skin from the lesion with fresh, predominantly small blisters. Early signs of pemphigus are intercellular edema of the epidermis and destruction of intercellular bridges (desmosomes) in the lower parts of the Malpighian layer. As a result of the loss of communication between epitheliocytes (acantholysis), first gaps are formed, and then bubbles, localized mainly suprabasally. The nasal cells, although they lose contact with each other, remain attached to the basement membrane. The bladder cavity usually contains rounded acantholytic cells with large hyperchromic nuclei and pale colored cytoplasm. Acantholysis can also be observed in the epithelial sheaths of hair follicles, where, as in the epidermis, gaps are formed, mainly above the basal layer. In old blisters occurs: regeneration of the epidermis, their bottom is covered with several layers of epithelial cells. In places of rejection of the lid of the bladder, its bottom is lined with cells of the basal layer. In the healing process, proliferation of the papillae of the dermis and elongation, sometimes significant, of the epidermal outgrowths are noted. In these cases, the histological picture resembles a vegetative pemphigus. In the dermis, inflammatory changes can be pronounced. The infiltrate consists of eosinophilic granulocytes, plasma cells and lymphocytes.

Similar changes are found on the mucous membranes. With damage to the oral mucosa, it is very difficult to excise a whole bladder, therefore, smears-imprints are used for diagnosis. in which, after staining according to the Romanovsky-Giemsa method, acantholytic cells are found (Tzank test). However, this test only supplements, but by no means replaces histological examination. Electron microscopic examination of the skin in the area of the bladder and in its clinically unchanged areas revealed the main changes in the area of intercellular contacts. In the initial stages of the formation of acantholysis, changes in the intercellular substance were revealed almost throughout the Malpighian layer, which leads to a loss of the ability to form desmosomal bonds. Cells that have lost contact with each other are rounded, the number of tonofilaments in them decreases. they concentrate around the nucleus, then undergo lysis and disappear.

The histogenesis of this type of pemphigus is the same as that of ordinary pemphigus.

Pemphigus foliaceus is characterized by a superficial arrangement of blisters, as a result of which they are clinically inconspicuous, quickly covered with scaly crusts, often layered due to the re-formation of blisters under them. The process is usually generalized, unlike ordinary pemphigus, it proceeds with an inflammatory reaction, which makes the lesions similar to exfoliative erythroderma, psoriasis, seborrheic dermatitis and other dermatoses. Mucous membranes are rarely affected. Nikolsky's symptom is sharply positive, with injury, extensive erosive surfaces appear. The prognosis for this form is less favorable than for pemphigus vulgaris.

Pathomorphology. In fresh foci, acantholysis usually occurs in the granular layer or directly below it with the formation of blisters of subcorneal localization. The phenomena of acantholysis can be both at the base and in the lid of the bladder. Sometimes, due to acantholysis, the stratum corneum and partly granular layer can separate without the formation of a bubble. In the circumference of the gap, epidermocytes do not have desmosomes and there is a tendency to separate them, as a result of which gaps can also form in the middle sections of the epidermis. It is possible to detach the entire epidermis above the basal layer. In older lesions with a more benign course of the disease, acanthosis, papillomatosis and hyperkeratosis are usually observed, sometimes with hyperkeratotic plugs at the mouths of the hair follicles. In areas of hyperkeratosis, pycnosis with heterochromia of individual cells can be observed, resembling "grains" in appearance in Darier's disease, in the dermis there is a moderately pronounced infiltrate, sometimes with the presence of eosinophilic granulocytes.

Histogenesis. Bubble formation in pemphigus foliaceus is also based on acantholysis, which occurs as a result of a pathological antigen-antibody reaction, but autoantibodies are directed against an antigen other than in the above types of pemphigus, namely desmoglein I, another important protein component of desmosomes in combination with placoglobin I In addition, the so-called eosinophilic spongiosis, which is detected in the epidermis in the earliest stages of the pathological process, sometimes before the development of acantholysis, morphologically resembling Dühring's herpetiform dermatitis, can play a role in the development of blisters. Electron microscopy during this period of the disease reveals dissolved intercellular cement and a reduced number of desmosomes. Tonofilaments are located perinuclearly, as in dyskeratosis. In this type of pemphigus, autoantibodies identical to those in pemphigus vulgaris are detected in the intercellular spaces of the epidermis.

Erythematous pemphigus is considered by us as a limited variant of pemphigus foliaceus, however, it is believed that this is an independent form of pemphigus or a combination of pemphigus with lupus erythematosus. This is indicated by clinical and morphological signs characteristic of both diseases. The lesions are located mainly on the back, chest and in the interscapular region. They have clinical signs of pemphigus (blisters), lupus erythematosus (erythema, sometimes atrophy) and seborrheic dermatitis (layering of scaly crusts) may also resemble impetigo, exudative psoriasis. Nikolsky's symptom is positive, changes in the mucous membranes are often observed.

Pathomorphology. Changes are similar to pemphigus foliaceus. In old elements, follicular hyperkeratosis with acantholysis and dyskeratosis in the granular layer is noted. With clinical similarity, lupus erythematosus is differentiated from erythematous pemphigus only histologically. Acantholysis and localization of the bubble in the granular layer of the epidermis, minor inflammatory infiltrates in the dermis in erythematous pemphigus distinguish it from lupus erythematosus.

Histogenesis. Changes in the epidermis. identified by electron microscopy are similar to those in pemphigus foliaceus, as is the autoimmune status. However, in this disease, direct immunofluorescence detects the luminescence of the basement membrane as a result of the deposition of immunoglobulin G in it, as well as antinuclear antibodies, which is characteristic of autoimmune diseases in general. th. Van Joost et al. (1984), conducting an immunomorphological study in seborrheic pemphigus, found that the pathogenesis of this disease is the primary defect in the function of T-suppressors, expressed in hyperproduction of autoantibodies.

differential diagnosis. Pemphigus vulgaris must be distinguished from other forms of pemphigus vulgaris, pemphigoid, Duhring's disease, and other bladder diseases.

In the clinical course of vegetative pemphigus, there are classical (Neumann type) and benign (Gallopo type) forms.

Symptoms. In the Neumann type, flabby blisters suddenly appear, as in the vulgar form, the tire of which quickly opens, exposing bright red erosions of an oval, round or irregular shape, tending to peripheral growth. Rashes often appear around natural openings and in folds (groin-femoral, intergluteal, axillary, under the mammary glands, in the navel). Over time (on the 5th-6th day), juicy, small, bright red vegetations with a fetid discharge form on the surface of the erosions. The number and size of vegetative erosions is increasing. Pustules may appear along the periphery of erosion. Nikolsky's symptom in most patients is positive.

In benign vegetative pemphigus (Gallopo type), lesions are predominantly located on intertriginous areas of the skin and less often on the mucous membranes of the mouth. The course of the disease is more favorable. This form is always accompanied by pustular and follicular elements, merging into infiltrated plaques with vegetations.

Histopathology. In the early stages of the disease, the histological picture in the area of blisters and erosions is similar to that observed in pemphigus vulgaris. Papillomatous and verrucous growths are characterized by the phenomena of papillomatosis and acanthosis with intraepidermal abscesses consisting of eosinophilic granulocytes. Immunomorphological studies in the intercellular spaces of the epidermis in patients reveal deposits of IgG.

differential diagnosis. Neumann's pemphigus vegetans must be differentiated from pemphigus vulgaris, secondary recurrent syphilis, drug toxicodermia (iododerma, bromoderma), the vegetative form of Darier's follicular dyskeratosis, and chronic familial benign Hailey-Hailey pemphigus.

Pemphigus foliaceus is much less common than common pemphigus.

Symptoms. Pemphigus foliaceus has characteristic specific features: the appearance of superficial flabby blisters with a tire race on unchanged or slightly hypersized skin. Their tire quickly ruptures even with a light touch or under the pressure of the bladder fluid. At the same time, juicy, bright red erosions are exposed with exudate, which soon shrinks into layered scales-crusts. Extensive eroded areas covered with layered crusts resemble exfoliative erythroderma. An important clinical sign of pemphigus foliaceus is the repeated, sometimes continuous, formation of superficial blisters under the crusts at the site of previous erosions.

Nikolsky's symptom (for the first time this symptom was described with a leaf-shaped form) is well expressed both near the lesions and in remote areas of the skin. As with other forms, the general condition of patients is disturbed (body temperature rises, a secondary infection joins, cachexia develops).

Histopathology. Histologically, in pemphigus foliaceus, acantholysis is noted, usually in the granular layer or under it (subcorneal fissures), acantholysis is pronounced. There is a pronounced inflammatory infiltrate in the dermis. In the intercellular spaces of the epidermis, antibodies of the IgG class are detected.

differential diagnosis. Pemphigus foliaceus must be differentiated from erythroderma of various origins (secondary erythroderma, toxicoderma), pemphigus vulgaris, Duhring's dermatitis herpetiformis (vesical form), toxic epidermal necrolysis of erythematous (seborrheic) pemphigus, etc.

Erythematous (seborrheic) pemphigus (Senier-Uscher syndrome) is one of the variants of true pemphigus, as evidenced by the frequent cases of its transition to ordinary or leaf-shaped pemphigus.

Symptoms. With erythematous pemphigus, symptoms of such dermatoses as erythematous lupus, pemphigus and seborrheic dermatitis occur.

As a rule, early rashes occur on the scalp, facial skin (in the area of the cheeks or on the back of the nose with the transition to the adjacent areas of the cheeks, forehead), later lesions appear on the trunk. There are erythematous foci with clear boundaries, on the surface of which there are thin or loose grayish scaly crusts. In the case of weeping, the lesions become covered with grayish-yellow or brownish crusts. Crusts result from the drying of the exudate of the blisters that form on the lesions or adjacent areas of the skin. Emerging bubbles are often not noticeable to both the patient and the doctor, because they are thin and flabby. They quickly open and become covered with dense or loose crusts, can merge with each other or remain isolated for a long time. Nikolsky's symptom is positive in most patients.

On the scalp, the rash may look like seborrheic dermatitis.

Mucous membranes are affected in about a third of patients. The course of the disease is long, with remissions.

Histopathology. Histopathological examination reveals cracks or blisters under the stratum corneum or granular layer of the epidermis as in pemphigus foliaceus. For erythematous pemphigus, follicular hyperkeratosis is often pathognomonic.

Direct immunofluorescence in the intercellular space of the epidermis in patients with erythematous pemphigus reveals fixed IgG.

differential diagnosis. Erythematous (seborrheic) pemphigus must be distinguished from lupus erythematosus, seborrheic eczema, ordinary, leaf-shaped, Brazilian pemphigus, subcorneal pustular dermatosis of Sneddon-Wilkinson.

Treatment of pemphigus

Since pemphigus is an autoimmune disease, its treatment should be exclusively pathogenetic. In this regard, modern treatment of patients with pemphigus is carried out with corticosteroid hormonal preparations and consists of two stages:

achievement of optimal results (complete cessation of new rashes, resolution of morphological elements) in a hospital;
long-term outpatient treatment with maintenance doses under careful dispensary supervision.

Corticosteroids are prescribed in large shock doses, depending on the severity and prevalence of the process, the weight of the patient. According to different authors, the dose is 1-2 mg/kg of the patient's weight. To reduce the dose and side effects of corticosteroids, as well as to increase the effectiveness of treatment, glucocorticosteroids are combined with methotrexate.

A combination of various schemes. Some authors recommend prescribing immunosuppressants after the therapeutic effect of corticosteroids has been achieved. Other authors prescribe methotrexate at the beginning of treatment once a week for 10-15 mg.

Immunosuppressants, in particular methotrexate, suppress the synthesis of antibodies, slow down allergic processes and have a nonspecific anti-inflammatory effect. Methotrexate (EBEWE) has the most favorable ratio of efficacy and tolerability compared to other cytostatics.

In the treatment of pemphigus, cyclosporine A (sandimmun-neoral) has a good effect. In this case, the initial dose of cyclosporine is 2.5 mg per 1 kg of body weight. Efficiency is increased when cyclosporine is combined with corticosteroids.

To increase the therapeutic effect of corticosteroids, systemic enzymes (phlogenzym, wobenzym) are added to the treatment. The dose depends on the severity of the disease and averages 2-3 tablets 3 times a day.

To restore disturbed protein, carbohydrate and fat metabolism, increase the activity of immunobiological processes and reduce decalcification, anabolic hormones - retabolil should be added to corticosteroid therapy. Retabolil also stimulates protein synthesis in the body.

Externally, aniline dyes, creams, ointments containing glucocorticosteroids and antibiotics, and agents that enhance epithelization are used.

To improve metabolic processes, microcirculation and epithelialization in lesions, some authors add laser therapy to basic therapy.

Since the blood serum of patients with active pemphigus contains antibodies to the intercellular substance of the epidermis and circulating immune complexes, plasmaphoresis, plasmasorption and hemosorption methods are used to remove these substances from the vascular bed.

Online Tests

Are you predisposed to breast cancer? (questions: 8)
In order to independently decide whether it is important for you to conduct genetic testing to determine mutations in the BRCA 1 and BRCA 2 genes, please answer the questions of this test...

True pemphigus

What is true pemphigus -

True (acantholytic) pemphigus- This is a chronic relapsing, hormone-dependent disease, during which a generalized spread of blisters occurs, as a result of which the general condition of the body suffers. The disease has a progressive course. Sometimes there may be periods of remission of varying severity and duration. Usually people 40-60 years old get sick.

What provokes / Causes of True pemphigus:

Causes and mechanisms of development pemphigus unknown, although there are many theories. These include: infectious, immunological, metabolic, neurogenic, endocrine, enzymatic, toxic. The most recognized cause by most researchers is immunopathological autoaggressive processes in the body. This is confirmed by the detection of antibodies against the intercellular substance in the affected skin, cystic fluid and blood serum of patients.

The use of the immunofluorescent method of examination confirms the presence of a luminescence characteristic only for patients with pemphigus in the region of the intercellular space in the spiny layer of the epidermis. It is most likely that the autoimmune mechanisms in pemphigus are pathogenetic rather than etiological.

Pathogenesis (what happens?) During Pemphigus True:

Some researchers consider all forms of pemphigus as types of a single disease that is caused by a filterable virus or a group of biologically related viruses. The authors of this theory give their evidence, such as:

infection of white mice and rabbits with the contents of the bubbles;
infection of chicken embryos with material from patients with pemphigus with the formation of bubbles in the embryos;
a positive complement fixation reaction prepared from the contents of the bladders and sera of patients with pemphigus and Dühring's dermatosis;
detection of virus-like formations in pemphigus cells during electron microscopy, identification of the cytopathogenic effect of blood serum and the contents of the blisters of patients with pemphigus and Dühring's dermatosis using the method of tissue culture and others. But all these data are not absolute evidence of the viral nature of the disease.

There is also n eurogenic concept of the occurrence of pemphigus, which is one of the first, which was supported and developed by P.V. Nikolsky. He considered pemphigus to be a neurotrophic disease caused by the degeneration of nerve cells. In favor of this theory, cases of the development of dermatosis after neuropsychic trauma, strong emotional upheavals are given. In some cases, patients who died from pemphigus showed degenerative changes in the cells of the spinal and medulla oblongata and spinal ganglia. These changes are likely to be pathogenic in nature.

exchange theory is based on significant changes in water, mineral and protein metabolism fixed in patients with pemphigus. Sodium chloride metabolism is regulated by the function of the adrenal cortex. This function is called mineralocorticoid. This theory has clinical evidence: the effectiveness of steroid hormones in the treatment of pemphigus, increased excretion of glucocorticoids in such patients, which confirms the oppression, up to the depletion of their glucocorticoid function of the adrenal cortex. For the endocrine concept of the etiology of pemphigus, there are known cases of the occurrence of this disease in pregnant women and its disappearance after childbirth. But it is most likely that metabolic and endocrine disorders are not primary in pemphigus, but occur secondary in response to the influence of some other factor. Among other things, there are isolated observations of the hereditary transmission of pemphigus.

Symptoms of Pemphigus Verus:

The existing classification distinguishes 4 forms of true pemphigus:

vulgar (usual);
vegetative;
leaf-shaped (exfoliative);
seborrheic.

True pemphigus is more common in women. The predominant age for the onset of pemphigus varies between 40 and 60 years. It is rare in childhood and adolescence and is characterized by a severe, often malignant course, up to death.

Pemphigus vulgaris (common)

Pemphigus vulgaris is the most common form of all forms of true pemphigus (approximately 75% of cases). With this type of dermatosis, the mucous membranes of the oral cavity and pharynx are first affected, and then the skin of the trunk, limbs, face, external genital organs, inguinal folds and armpits are involved in the process. The disease has a severe chronic course. A sudden onset is characteristic, when, for no apparent reason, against the background of unchanged skin, initially tense, and then quickly becoming flaccid, small blisters appear. In the initial stages of the disease, they are filled with a clear serous fluid, which later becomes cloudy. Under the bubbles, weeping erosions of various shapes quickly form. The healing of eroded surfaces ends with the formation of layered brown crusts, after falling off of which brownish pigmentation remains.

There are cases of spontaneous improvement (without treatment), which are replaced by exacerbations of the disease. If the course of pemphigus is benign, then the general condition of the patient practically does not change. The general serious condition and exhaustion of the body, septic fever, which is especially pronounced when a secondary infection is attached, is observed in the malignant course of the disease. In these cases, eosinophilia is expressed in the blood of patients, the protein content is reduced, protein fractions containing immunoglobulins A, J, M are changed. Damage to the liver, kidneys, heart and lungs is quite common. With pemphigus, there is almost no itching. Numerous erosions are painful and aggravated by changing the position of the body, dressings. Lesions cause especially excruciating pains when they are localized on the mucous membranes of the oral cavity, genitals, etc.

An important clinical and diagnostic sign of pemphigus is a symptom, or phenomenon, Nikolsky. It is caused by degeneration in the Malpighian layer of the skin, the so-called "acantholysis", and consists in the fact that when pulling on a piece of bladder covers, the epidermis detaches on apparently healthy skin. Also, when the skin, which seems healthy and is located between the blisters, is rubbed, there is a slight rejection of the upper layers of the epidermis.

There is a diagnostic test for pemphigus called the Asboe-Hansen sign. It lies in the fact that when pressing on the bubble that has not yet opened with a finger, one can see how the liquid in the bubble exfoliates the adjacent areas of the epidermis and the bubble itself increases in size along the periphery before our eyes. But Nikolsky's symptom for true pemphigus is not a strictly obligatory sign of only this disease, although it is of great value for diagnosis. This symptom is also found in other dermatoses: congenital epidermolysis bullosa, Ritter's disease, Lyell's syndrome. In almost all patients with pemphigus, this symptom is positive only in the acute phase. In other periods of the disease, it is negative.

Well-chosen treatment of patients with true pemphigus significantly lengthens the periods of remission (improvement) and delays the onset of relapse, and maintenance therapy with the use of steroid hormones saves lives for many years.

Vegetative pemphigus

This form of pemphigus at the initial stages of development is clinically similar to pemphigus vulgaris and often begins with the appearance of blisters on the mucous membranes of the oral cavity. But with vegetative pemphigus, there is a tendency for blisters to be located around natural openings, the navel, and also in the area of large skin folds (axillary, inguinal-femoral, intergluteal, under the mammary glands, behind the auricles). When the blisters open, erosions form in their place, covered with a dirty coating and papillomatous growths. In this case, a large amount of exudate is released. There is a tendency to confluence of lesions and the formation of extensive vegetative surfaces, in some places with purulent-necrotic decay. Nikolsky's symptom is often positive. Patients feel pain and burning, which make active movements difficult.

With successful treatment, the vegetation is compacted, dried, epithelization of erosions occurs with pronounced residual pigmentation. But some patients can quickly develop exhaustion, and some of them die.

Leaf-shaped (exfoliative) pemphigus

This variety of pemphigus is characterized by a sharp acantholysis, which leads to the formation of superficial cracks, which later turn into blisters. In adults, pemphigus foliaceus is more common than vegetative, and in children it predominates over other types of pemphigus vulgaris.

The disease begins on apparently unchanged skin with the appearance of flabby, thinly covered blisters that protrude slightly above the surface. The opening of the bubbles takes place quite quickly, and extensive erosion forms in their place. But most often the tires of the bubbles shrink, forming thin lamellar scales-crusts. Under the crusts, erosion epithelialize slowly. Layered crusts are formed due to the formation of new portions of exudate (detachable) and resemble puff pastry, therefore this type of pemphigus is called "leaf-like" - exfoliative.

Symptom P. V. Nikolsky, described in 1896, it is always sharply positive with pemphigus foliaceus. The disease has a long-term chronic course with periods of spontaneous improvement. As a result of progression, the process gradually occupies large areas of the skin, up to the scalp, and often proceeds as erythroderma. The mucous membranes of the oral cavity are extremely rarely affected. The severity of the general condition of the patient depends on the extent of skin lesions. If the foci are few in number, then the condition is disturbed little, and with generalized lesions, an increase in temperature, disturbances in water and salt metabolism, progression of anemia, and eosinophilia are noted. Patients in such severe cases weaken, lose weight, up to the development of cachexia.

Seborrheic (erythematous) pemphigus - Senier-Uscher syndrome

This disease is true pemphigus, as it can turn into a leaf-shaped or vulgar form.

The course of seborrheic pemphigus long-term, but in most cases benign. Most often, the skin of the face, chest and back is first affected. Sometimes the process affects the scalp. Closely spaced fatty crusts are formed on reddened skin, i.e., on an erythematous background. The process on the face is similar in appearance to the picture of scarring erythematosis.

Wet erosions are found under the crusts. If a smear-imprint is made from these eroded surfaces, then anatomical cells can be detected in it. Quite often, the blisters are invisible, and as a result, a false idea is created about the primacy of the crusts. On the mucous membranes, seborrheic pemphigus rarely appears, but if the pathological process develops on them, then this is a poor prognostic sign.

This type of pemphigus is characterized by intraepidermal abscesses (intradermal ulcers), which consist of eosinophils (leukocytes).

Diagnosis of true pemphigus:

If a disease proceeds classically, the diagnosis is not difficult. It is necessary to differentiate pemphigus from erythema multiforme exudative, bullous toxicoderma, Duhring's dermatosis herpetiformis, congenital epidermolysis bullosa, and bullous lupus erythematosus.

Pemphigus vera treatment:

Steroid hormones are prescribed according to the scheme. First treatment carried out in shock doses. Prednisolone or methylprednisolone - 40-60 mg each, triampcinolone - 32-48 mg each, dexamethasone - 4-6 mg per day. If the appearance of new rashes stops, the effects of exudation in the lesions decrease, the dose of hormones is reduced, but this is done very slowly in order to avoid exacerbation of the process and in order to prevent the “withdrawal phenomenon” of steroid hormones.

The maintenance dose of hormones is prescribed strictly individually for a particular patient, so that it is minimal and does not lead to a relapse of the disease. Such treatment is carried out for a long time. To reduce the dose of corticosteroid hormones and avoid severe complications caused by them, hormones are combined with the simultaneous administration of cytostatics - immunosuppressants, which include: methotrexate, prospidin, azathioprine. Anabolic hormones are also used - methylandrostinolone, nerobolil, methylandrostenediol, calcium, potassium preparations, agents for increasing phagocytosis (pentoxyl, methyluracil, ascorbic acid, vitamins, rutin, ascorutin, riboflavin, calcium pantothenate, folic acid). In case of secondary infection, antibiotics are used.

Of great importance symptomatic therapy, which depends on the general condition of the patient - these are blood transfusions, infusions of native plasma, the introduction of y-globulin, liver preparations (heparin, vitagepancreatin, Hepavit tablets), autohemotherapy.

Patients should follow a diet balanced in terms of protein, calcium and vitamins. If the patient has large lesions of the skin and mucous membranes, frequent warm baths with disinfectant solutions, for example, with potassium permanganate, are prescribed, followed by the use of disinfectants: Castellani liquid, 1-2% aqueous solution of pyoctanin, 5% boron naphthalene ointment, etc. The affected areas of the skin are lubricated with corticosteroid ointments ("Locacorten", "Flucinar", "Dermazolone", "Oxycort", "Gioksizon", etc.).

Which doctors should you contact if you have Pemphigus Verus:

Are you worried about something? Do you want to know more detailed information about True Pemphigus, its causes, symptoms, methods of treatment and prevention, the course of the disease and diet after it? Or do you need an inspection? You can book an appointment with a doctor– clinic Eurolaboratory always at your service! The best doctors will examine you, study the external signs and help identify the disease by symptoms, advise you and provide the necessary assistance and make a diagnosis. you also can call a doctor at home. Clinic Eurolaboratory open for you around the clock.

How to contact the clinic:
Phone of our clinic in Kyiv: (+38 044) 206-20-00 (multichannel). The secretary of the clinic will select a convenient day and hour for you to visit the doctor. Our coordinates and directions are indicated. Look in more detail about all the services of the clinic on her.

(+38 044) 206-20-00

If you have previously performed any research, be sure to take their results to a consultation with a doctor. If the studies have not been completed, we will do everything necessary in our clinic or with our colleagues in other clinics.

You? You need to be very careful about your overall health. People don't pay enough attention disease symptoms and do not realize that these diseases can be life-threatening. There are many diseases that at first do not manifest themselves in our body, but in the end it turns out that, unfortunately, it is too late to treat them. Each disease has its own specific signs, characteristic external manifestations - the so-called disease symptoms. Identifying symptoms is the first step in diagnosing diseases in general. To do this, you just need to several times a year be examined by a doctor not only to prevent a terrible disease, but also to maintain a healthy spirit in the body and the body as a whole.

If you want to ask a doctor a question, use the online consultation section, perhaps you will find answers to your questions there and read self care tips. If you are interested in reviews about clinics and doctors, try to find the information you need in the section. Also register on the medical portal Eurolaboratory to be constantly up to date with the latest news and information updates on the site, which will be automatically sent to you by mail.

Other diseases from the group Diseases of the skin and subcutaneous tissue:

Abrasive pre-cancer cheilitis of Manganotti

actinic cheilitis

Allergic arteriolitis or Reiter's vasculitis

Allergic dermatitis

skin amyloidosis

Anhidrosis

Asteatosis, or sebostasis

Atheroma

Basalioma of the skin of the face

Basal cell skin cancer (basalioma)

Bartholinitis

White piedra (knotty trichosporia)

Warty skin tuberculosis

Bullous impetigo of newborns

Vesiculopustulosis

Freckles

Vitiligo

Vulvitis

Vulgar or strepto-staphylococcal impetigo

Generalized rubromycosis

Hydradenitis

Hyperhidrosis

Hypovitaminosis of vitamin B12 (cyanocobalamin)

Vitamin A hypovitaminosis (retinol)

Hypovitaminosis of vitamin B1 (thiamine)

Hypovitaminosis of vitamin B2 (riboflavin)

Hypovitaminosis of vitamin B3 (vitamin PP)

Vitamin B6 hypovitaminosis (pyridoxine)

Vitamin E hypovitaminosis (tocopherol)

hypotrichosis

Glandular cheilitis

Deep blastomycosis

Fungal mycosis

Epidermolysis bullosa group of diseases

Dermatitis

Dermatomyositis (polymyositis)

Dermatophytosis

splinters

Malignant granuloma of the face

Itching of the genitals

Excess hair, or hirsutism

Impetigo

Indurative (compacted) Bazin's erythema

Ichthyosis and ichthyosis-like diseases

Skin calcification

Candidiasis

Carbuncle

Pilonidal cyst

Skin itching

granuloma annulare

contact dermatitis

Hives

Red grainy nose

Lichen planus

Palmar and plantar hereditary erythema, or erythrosis (Lahn's disease)

Skin leishmaniasis (Borovsky's disease)

Lentigo

liveoadenitis

Lymphadenitis

Fusk line, or Andersen-True-Hackstausen syndrome

Lipoid necrobiosis of the skin

Lichenoid tuberculosis - lichen scrofulous

Riehl melanosis

skin melanoma

Melanoma dangerous nevi

Meteorological cheilitis

Mycosis of nails (onychomycosis)

Mycoses of the feet

Multimorphic exudative erythema

Mucinous alopecia of Pinkus, or follicular mucinosis

Hair growth disorders

Neacantholytic pemphigus, or scarring pemphigoid

Pigmentation incontinence, or flea-sulzberger syndrome

Neurodermatitis

Neurofibromatosis (Recklinghausen's disease)

Baldness or alopecia

Burn

burns

frostbite

Papulonecrotic tuberculosis of the skin

Inguinal epidermophytosis

Periarteritis nodular

Pint

Pioallergides

pyoderma

Squamous cell skin cancer

Superficial mycosis

tardive cutaneous porphyria

Polymorphic dermal angiitis

Porfiria

graying hair

Scabies

Occupational skin diseases

Manifestation of vitamin A hypervitaminosis on the skin

The manifestation of hypovitaminosis of vitamin C on the skin

Skin manifestations of herpes simplex

Pemphigus is a group of skin diseases, which are characterized by the spontaneous appearance of blisters on the skin or mucous membranes, prone to generalization and fusion. Distinguish the true form of pemphigus, a synonym is pemphigus. Pemphigoid, a group of clinically similar dermatosis, also occurs.

External signs of pemphigus of any type have much in common. But the varieties differ in etiology, have pathogenetic and clinical features, and need different therapy.

The reasons

Pemphigus occurs against the background of acantholysis - the destruction of intercellular bonds in the epidermis by antibodies, which causes the formation of blisters. This is due to the pathological reaction of the immune system against the background of a decrease in the body's defenses. The exact cause of dermatosis has not been established.

In pemphigoid forms, connections between cells are preserved, but excess intercellular fluid pushes the cells apart, forming a vesicular rash. They are often called epidemic variants of skin lesions, due to the role of viruses and bacteria in etiology. But the autoimmune factor in the development of the disease is also present.

How is epidemic pemphigus transmitted? A person becomes infected with a viral form during epidemics of respiratory infections. In this case, enteroviruses are transmitted by airborne droplets. If the rules of personal hygiene are not observed, the infection can be transmitted by contact-household, fecal-oral methods.

You can become infected through contact with people from other regions and on trips abroad, when a person encounters an unfamiliar microbial background.

Among bacteria, staphylococci and streptococci are dangerous. Most often, babies are affected in the first 10 days of life. Risk factors include: prematurity, birth trauma. Also, the infectious form of pemphigus can be transmitted in the presence of pyoderma in medical personnel.

There are factors that increase the risk of developing any variant of vesicular dermatosis:

hereditary predisposition;
autoimmune disorders;
decreased immune defense;
diabetes;
oncological diseases;
chronic diseases of various organs and systems in the stage of exacerbation or decompensation.

Symptoms

What does pemphigus look like in various forms? The viral variety is contagious, but the disease manifests itself after a week. On the mucous membranes, painful yellowish vesicles are formed, surrounded by a halo of inflammation. There is a fever, bad breath. The characteristic symptoms of viral pemphigus are lesions of the skin of the hands and feet.

Pemphigus on the palms has the following clinical features:

unchanged skin background;
elongated vesicles 3-5 mm in diameter, painful when touched;
skin itching in the area of rashes;
inflamed border around the vesicles.

Pemphigus on the hands usually does not leave marks, but there may be unstable pigmentation.

The epidemic form of pathology in newborns is characterized by the appearance of vesicles with purulent contents on the skin, which rapidly increase in number against the background of a deterioration in the condition.

Pemphigus vulgaris, or vulgaris, begins with the appearance of a single bubble, sometimes reaching the size of a walnut. Then multiple vesicles appear. Their contents gradually become cloudy, an admixture of blood is possible. With pemphigus in adults, the symptoms of intoxication are pronounced, there is no itching and pain in the places of rashes.

In all forms, after the opening of the blisters, painful erosions are formed, gradually covered with crusts.

Kinds

There are several main types of pemphigus. The most common form is pemphigus vulgaris, other variants are rare.

ordinary

Pemphigus vulgaris often affects middle-aged people against the background of a malfunction of the immune system. It can proceed in a malignant form, resistant to all modern methods of treatment.

In advanced cases, the pathology leads to death.

vegetative

With this form, the blisters quickly burst, and gradually increasing and often bleeding vegetations are found on the exposed bottom.

Foliate

Pemphigus of this variety is rare in adults, children are more often ill. At first, the skin turns red, resembling a picture of eczema, then flat and flabby vesicles appear. It quickly turns into large-lamellar dermatosis with areas of erythema and erosion. The resulting layered crusts resemble leaves, hence the name of the form.

Erythematous

Another name is seborrheic. This is due to the fact that the first foci of pemphigus appear on the head. Small bubbles appear, which, drying up, turn into yellowish-gray crusts. Under them, erosion is found.

Brazilian

This form of pemphigus is endemic. It is believed that the main cause of its development is a virus. Occurs at any age. Gives frequent relapses and complications.

Localization

Pemphigus vulgaris affects the armpits, groin area, skin of the back, chest, palms - very rarely.

Foci of the vegetative form are located on the mucous membranes of the mouth, the skin of the lips, axillary and inguinal folds, around the navel, in the perianal region, under the mammary glands.

Leaf-shaped pemphigus is found on the scalp, skin of the back and chest.

Foci of erythematous pemphigus are localized on the face, scalp, less often on the skin of the trunk.

Manifestations of the enterovirus form are most often found on the palms, soles, sometimes on the mucous cheeks, tongue.

Which doctor treats pemphigus?

Pemphigus is treated by dermatologists and infectious disease specialists. With the first signs of pathology, they often turn to dentists, finding rashes in the mouth. The dentist, after examining the patient, should refer him to a specialized specialist.

How to define pathology?

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

histology;
cytology;
immunogram.

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

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

Methods of therapy

How to treat pemphigus? It depends on its etiology, form, severity of the course and the state of health of the patient. Treatment of pemphigus in adults should begin immediately, therapy is complex, long and persistent.

The following groups of drugs are used:

Glucocorticosteroid hormones. More often intramuscularly or intravenously use Prednisolone.
Immunosuppressors - Methotrexate, Sandimmun.
Antibiotics. With microbial etiology and secondary infection.
Calcium, potassium, sodium preparations - for the prevention of electrolyte imbalance.
Anabolic hormones - to prevent unwanted (catabolic) effects of glucocorticoids.
In severe cases, infusion therapy, hemodialysis are used.

Treatment for pemphigus involves the use of local remedies. Betamethasone, solutions and ointments are prescribed to prevent suppuration and accelerate healing.

Treatment of the viral form

This type of dermatosis involves a combination of etiological and symptomatic therapy:

antiviral drugs (Viferon) in the form of suppositories, tablets, injections;
desensitizing agents for skin itching (Suprastin and locally Fenistil-gel);
anti-inflammatory drugs for fever and pain (Paracetomol);
adherence to a diet with the exception of spicy and hot dishes;
rinsing the mouth with solutions of anesthetics and antiseptics (Strepsis spray, Chlorhexidine), infusions of anti-inflammatory herbs.

How to cure pemphigus on the hands? Against the background of systemic therapy, brilliant greens, antibacterial and wound-healing ointments, lotions with nettle decoction, aloe juice are applied topically.

Complications

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

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

The disease, which is severe, requires the use of potent drugs. Due to the need for long-term hormone therapy, the following problems arise:

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

Prevention

Since there are two mechanisms for the development of forms of vesicular dermatosis - autoimmune and infectious, it is recommended to adhere to the following preventive measures:

eliminate physical and nervous overload, excessive insolation, including a visit to the solarium;
the diet should be balanced, contain a sufficient amount of proteins, vitamins and minerals;
when in contact with children, observe all hygiene rules;
if there is a sick person in the family, it is necessary to limit his contacts with other family members, especially with children;
teach your child to use only their own dishes, linen and toys.

Pemphigus in adults is characterized by a long course, a tendency to relapse, the likelihood of serious complications, up to death. Therefore, it is required to begin treatment immediately, since a rapid deterioration in the condition is possible.

What is true pemphigus
Symptoms of pemphigus verus
Pemphigus vera treatment
Which Doctors Should You See If You Have True Pemphigus?

What is true pemphigus

What provokes True pemphigus

Pathogenesis (what happens?) during Pemphigus vera

infection of white mice and rabbits with the contents of the bubbles;
infection of chicken embryos with material from patients with pemphigus with the formation of bubbles in the embryos;
a positive complement fixation reaction prepared from the contents of the bladders and sera of patients with pemphigus and Dühring's dermatosis;
detection of virus-like formations in pemphigus cells during electron microscopy, identification of the cytopathogenic effect of blood serum and the contents of the blisters of patients with pemphigus and Dühring's dermatosis using the method of tissue culture and others. But all these data are not absolute evidence of the viral nature of the disease.

Symptoms of pemphigus verus