Pemphigus vulgarisis a disease with an autoimmune mechanism of development, characterized by the appearance of blisters on the mucous membranes and skin, which then open, turning into bright pink erosions. With Pemphigus vulgarisis, there is often an increase and fusion of erosions with the formation of significant lesions in the area. Diagnosis is carried out by biopsy of a newly appeared bladder, its histological and immunological examination. Corticosteroids, cytostatics, extracorporeal hemocorrection methods are used in the treatment of Pemphigus vulgarisis.
General information
Pemphigus vulgarisis is the most common clinical form of pemphigus. The incidence of it in the world is 0.1-0.5 per 100,000 population. Usually people aged 30-60 years get sick. Like other forms of pemphigus, pemphigus vulgarisis refers to bullous dermatoses, since its main element is a bubble. About 2/3 of cases of the disease begin with the appearance of blisters on the oral mucosa and only a few months later the skin is involved in the process.
Causes
The development of pemphigus vulgarisis is associated with disorders in the immune system, as a result of which IgG-type antibodies are produced to the own cells of the spiny layer of the epidermis. Under the action of autoantibodies, desmosomes that bind epidermal cells to each other are destroyed. The loss of connections between cells (acantholysis) leads to the fact that the space between them is filled with intercellular fluid with the formation of acantholytic bubbles characteristic of pemphigus vulgarisis.
Symptoms
Most often, pemphigus vulgarisisbegins with the mucous membrane of the mouth and pharynx. Due to mechanical damage caused by food, the bubbles open so quickly that they can almost never be seen. Bright red painful erosions form in the mouth against the background of unchanged mucosa. Fragments of a burst bubble covering erosion create a picture of a whitish plaque on it, but they are easily removed with a spatula. Gradually, the number of erosions increases. Without specific therapy, they do not heal, but on the contrary grow and merge. Due to the pronounced pain syndrome, the patient cannot eat and talk. There is a fetid smell from the mouth.
The appearance of blisters on the skin with pemphigus vulgarisis may occur several months after their occurrence in the oral cavity, but may also occur at the beginning of the disease. Blisters form on externally unchanged skin, they are filled with a clear liquid and are often not accompanied by itching or pain. In some cases, redness of the skin in the form of a thin rim is observed around the blisters. Pemphigus vulgarisis is characterized by the focal appearance of rashes in various parts of the body. Most often, the chest, back, axillary areas and inguinal folds are affected. Over time, new blisters occur with the involvement of previously healthy skin areas in the process.
A few days after their appearance, the bubbles open. The resulting erosion has a bright pink color. Gradually increasing in diameter, they merge and occupy vast areas of the skin. The general condition of the patient with pemphigus vulgarisis, which is not disturbed at first, becomes worse, subfebrility and weakness occur. Pronounced pain sensations prevent active movements. When an infection is attached, pyoderma develops: the fluid in the bubbles becomes cloudy, erosions are covered with purulent discharge, and the patient’s condition deteriorates sharply. Increasing cachexia or sepsis (when an infection is attached) can lead to the death of the patient.
Diagnosis
In the clinical diagnosis of pemphigus vulgarisis, mechanical symptoms indicating acantholysis are important. These include Nikolsky’s symptom — peeling of the epidermis with slight friction of a healthy-looking skin area. The marginal symptom of Nikolsky is checked by pulling on a piece of the wall of a burst bubble. If it is positive, then the epidermis is exfoliated at a fairly large distance from erosion. Asbo-Hansen’s symptom — pressing a finger on the bladder with pemphigus vulgarisis leads to peeling of the epidermis along the periphery of the bladder and an increase in its area.
To confirm the diagnosis of pemphigus vulgarisis, a cytological examination is performed using the Tzanka method. Microscopy of a smear print obtained from the bottom of erosion reveals characteristic acantholytic cells in the spiny layer of the epidermis. For histological examination during a biopsy, a skin area containing a fresh bubble is taken. The study detects a cavity located inside the epidermis and determines the mechanism of its occurrence.
Additional in the diagnosis of pemphigus vulgarisis are immunological methods that confirm or refute the autoimmune nature of the disease. A direct immunofluorescence reaction (RIF) reveals IgG accumulations in the intercellular space and on the membranes of epidermal cells. Indirect RIF is performed with the patient’s serum and determines the presence of antibodies to desmosomes of epidermal cells in it.
Treatment and prognosis
The only way to save a patient with pemphigus vulgarisis is to prescribe high doses of corticosteroids: dexamethasone, triamcinolone and prednisolone. The initial dose of the drug depends on the severity of the patient’s condition. Dosage reduction begins only after epithelization of all erosions and the cessation of fresh rashes. Gradually, a maintenance dose is selected — the minimum daily amount of the drug, when taking which there is no appearance of new rashes. The patient should take this dose constantly.
Cytostatics are used together with corticosteroid therapy: methotrexate, cyclosporine, azathioprine. This makes it possible to treat pemphigus vulgarisis with smaller doses of corticosteroids and achieve remission of the disease faster. The use of extracorporeal hemocorrection methods (plasmophoresis, hemosorption, etc.) makes it possible to purify the blood from the immune complexes and antibodies circulating in it. This contributes to the remission of vulgar pemphigus and is especially relevant with weak efficacy of corticosteroid therapy. In the treatment of pemphigus vulgarisis, potassium preparations, anabolic steroids are used, and when an infection is attached, antibiotics are used. To prevent complications of corticosteroid therapy, medications that protect the stomach wall (bismuth nitrate, etc.) and other drugs are prescribed.
Timely treatment of vulgar pemphigus with corticosteroids, as a rule, avoids a fatal outcome. However, long-term use of these drugs inevitably leads to severe complications from internal organs and systems, which in turn can cause the death of the patient.
