Vulgar puzyrchatka (a puzyrchatka ordinary) — the disease with the autoimmune mechanism of development which is characterized by emergence on mucous membranes and skin of bubbles which then are opened, turning into bright pink erosion. At a vulgar puzyrchatka often there is an increase and merge of erosion to formation of the defeat centers, considerable on the area. Diagnostics is carried out by a biopsy of recently appeared bubble, its histologic and immunological studying. In treatment of a vulgar puzyrchatka corticosteroids, cytostatics, methods of extracorporal haemo correction are applied.
Vulgar puzyrchatka — the most frequent clinical form of a puzyrchatka. Incidence of it in the world makes 0,1-0,5 on 100000 population. Usually aged people of 30-60 years are ill. As well as other forms of a puzyrchatka, the vulgar puzyrchatka belongs to a bullous dermatosis as her basic element is a bubble. About 2/3 cases begin with emergence of bubbles on a mucous oral cavity and only several months later the integument is involved in process.
Causes of a vulgar puzyrchatka
Development of a vulgar puzyrchatka is connected with violations in work of immune system from which development of antibodies like IgG to own cages of a shipovaty layer of epidermis results. Under action autoantitet there is a destruction of the desmosomes connecting cells of epidermis among themselves. Loss of communications between cages () leads to the fact that the space between them is filled with intercellular liquid with formation of akantolitichesky bubbles, characteristic of a vulgar puzyrchatka.
Symptoms of a vulgar puzyrchatka
The most often vulgar puzyrchatka begins with mucous a mouth and a pharynx. Because of mechanical damage by food bubbles are opened so quickly that they practically never manage to be seen. In a mouth against the background of not changed mucous bright red painful erosion are formed. The scraps of the burst bubble covering an erosion create a picture of a whitish raid on it, however they without difficulty act the pallet. Gradually the quantity of erosion increases. Without specific therapy they do not begin to live, and on the contrary grow and merge. Because of the expressed pain syndrome the patient cannot eat and talk. The fetid smell from a mouth is noted.
Emergence of bubbles on skin at a vulgar puzyrchatka can happen in several months after their emergence in a mouth, but can be observed also at the beginning of a disease. Bubbles are formed on externally not changed skin, they are filled with transparent liquid and often are not followed by either an itch, or pain. In some cases around bubbles reddening of skin in the form of a thin rim is observed. Focal emergence of rashes in various parts of the body is characteristic of a vulgar puzyrchatka. Most often the breast, a back, axillary areas and inguinal folds is surprised. Over time there is an emergence of new bubbles to involvement in process of sites of skin healthy earlier.
In several days after the emergence bubbles are opened. The erosion which are formed at the same time have bright pink coloring. Gradually increasing in the diameter, they merge and occupy extensive sites of an integument. The general condition of the patient with a vulgar puzyrchatka which is not broken at first is worse to become, arises subfebrilitt also weakness. The expressed pain obstructs the active traffic. At accession of an infection, the piodermiya develops: the liquid which is in bubbles becomes muddy, erosion become covered purulent separated, and the condition of the patient sharply worsens. The accruing kakheksiya or sepsis (at accession of an infection) can lead to death of the patient.
Diagnostics of a vulgar puzyrchatka
In clinical diagnostics of a vulgar puzyrchatka the mechanical symptoms testifying to an akantoliza matter. Nikolsky's symptom — flaking of epidermis at easy friction of the site of skin healthy by sight concerns to them. The regional symptom of Nikolsky is checked by a pandiculation for a scrap of a wall of the burst bubble. If it is positive, then there is a flaking of epidermis on quite long distance from an erosion. Asbo-Hansen's symptom — pressing by a finger on a bubble at a vulgar puzyrchatka leads to flaking of epidermis on the periphery of a bubble and increase in its area.
For confirmation of the diagnosis of a vulgar puzyrchatka conduct a cytologic research on Ttsank's method. The microscopy of dab print received from an erosion bottom reveals characteristic akantolitichesky cages in a shipovaty layer of epidermis. For a histologic research during a biopsy take the site of skin containing a fresh bubble. The research finds the cavity located in epidermis and defines the mechanism of its emergence.
In diagnostics of a vulgar puzyrchatka the immunological methods confirming or disproving the autoimmune nature of a disease are additional. Direct reaction of immunofluorescence (REEF) reveals the congestions IgG in intercellular space and on covers of cells of epidermis. Indirect the REEF is carried out with serum of the patient and defines existence of antibodies in it to desmosomes of cells of epidermis.
Treatment and forecast of a vulgar puzyrchatka
The only way to save sick with a vulgar puzyrchatka consists in purpose of high doses of corticosteroids: dexamethasone, triamtsinolon and Prednisolonum. The initial dose of medicine depends on weight of a condition of the patient. Decrease in a dosage is begun only after an epitelization of all erosion and the termination of fresh rashes. Gradually select the supporting dose — the minimum daily quantity of medicine at which reception emergence of new rashes is not observed. Has to accept this dose of the patient constantly.
Together with kortikosteroidny therapy apply cytostatics: methotrexate, , . It allows to carry out treatment of a vulgar puzyrchatka by smaller doses of corticosteroids and quicker to achieve remission of a disease. Application of methods of extracorporal haemo correction (, haemo sorption, etc.) gives the chance to purify blood of the immune complexes and antibodies circulating in it. It promotes remission of a vulgar puzyrchatka and is especially urgent at weak efficiency of kortikosteroidny therapy. In treatment of a vulgar puzyrchatka use potassium medicines, anabolic steroids, at accession of an infection — antibiotics. For prevention of complications of kortikosteroidny therapy appoint the medicines protecting a stomach wall (bismuth nitrate, etc.), and other medicines.
In due time begun therapy of a vulgar puzyrchatka corticosteroids, as a rule, allows to avoid a lethal outcome. However long-term reception of these medicines inevitably leads to emergence of heavy complications from internals and systems which in turn can become a cause of death of the patient.