True pemphigus is one of the diseases of the dermis. Dermatologists refer this pathology to autoimmune, which has a chronic character. There is a pathology in the form of blisters, they cover the dermis, mucous membranes.
Features of the disease
The disease described in the article is also known among physicians called acantholytic / bullous dermatitis. Bubbles arise due to acantholysis( exfoliation).
The disease is chronic. The patient may have remissions with different duration, nature of expression.
This prevalence of the disease is considered by some scientists to be the result of consanguineous marriages that are tolerated by certain peoples. According to the scientific literature, women are more prone to infection with this form of dermatosis. But in practice, the disease is equally common among both sexes, whose age ranges from 40 to 60 years. Very rarely it is diagnosed in children.
Pemphigus, true pemphigus( photo)
Pemphigus classification
Acantholytic pemphigus has several separate forms, each of them has its own special features:
- leaf-shaped;
- seborrhoeic;
- is vulgar;
- is vegetating.
Causes of
The etiology of this pathology is not fully understood. Scientists put forward 2 theories on account of its origin:
- viral;
- is an autoimmune disease.
Modern scientists have proven the leading role of autoimmune processes. Their manifestation is considered a response to the changes in the antigenic structure of the dermis cells. Such a reaction is provoked by various damaging agents.
We indicate the factors that affect cell damage:
- biological;
- physical;
- chemical.
Symptoms of
Each of the clinical forms of the pathology studied is characterized by a chronic undulating course. General deterioration is noted in the absence of appropriate therapy. Let's consider the signs peculiar to each of the forms of a true pemphigus.
Vulgary
It is characterized by bubbles of different sizes, the skin on the bladder is rather thin, sluggish. Under the skin is a serous fluid. Bubbles appear on visually healthy dermis, mucous membranes of the genitals, mouth, nasopharynx, oropharynx.
Initially, the rash covers the mucous membranes( mouth, pharynx, nose, lip rims).Patients can manifest such pathologies:
- rhinitis;
- stomatitis;
- laryngitis;
- gingivitis.
Characteristic signs of this form of pathology are:
- hypersalivation;
- pain when swallowing;
- appearance of a specific odor in the mouth;
- pain when talking.
After 3-6 months, the infection spreads to the skin. Bubbles remain intact for a short time because of a very thin coating. They burst very quickly, after them on the dermis, only the wounds that cause pain are noticeable. Sources of bubbles may crumble. Erosion is characterized by peripheral growth, with the condition of the patient significantly deteriorating. There may be intoxication, to join a secondary infection. Doctors detect the symptom of Nikolsky( with a weak mechanical effect, separation of the epithelial layer is noted).
Sheet
Thin-walled bubbles appear continuously, they appear in the same place. After bursting, the bladder leaves the wounds, they form crusts. The crusts can be massive due to the drying out of the exudate. Mucous disease does not affect.
This pathology is characterized by:
- formation of flat bubbles;
- is a positive symptom of Nikolsky;
- merging of bubbles;
- development of exfoliative erythroderma;
- appearance of wounds;
- formation of layered crusts;
- sepsis;
- deterioration;
- development of cachexia.
Seborrheic
Seborrheic( Senira-Asher syndrome) occurs initially on the seborrhoeic areas of the body:
- face;
- back;
- the scalp;
- chest.
The area of the lesion is separated by clear boundaries. On the dermis are visible crusts( yellow, brownish-brown).They arise after the sifting of small bubbles with a thin, flabby skin. After the burst, which occurs very quickly, the wound remains. The disease is inherent in the symptom of Nikolsky. After long periods of time( months, years), pathology passes to new areas of the body. Sometimes it covers the mucous membranes( may appear in the mouth).
Vegetating
Vesicating pemphigus. It can remain for many years in the form of benign pathology, occupying extremely limited areas of the dermis. The patient's state of health remains in satisfactory condition. Blisters arise in the mouth, on the genitals, in the nose, on the skin folds.
The bottom of the wound is covered with a purulent coating, on the periphery there are pustules. Near the focus of infection will be a positive symptom of Nikolsky.
Diagnosis
General rules
- The diagnosis is based on external signs of pathology.
- Specialists consider the presence of Nikolsky's symptom.
- Direct reaction of immunofluorescence is very important.
- Cytological examination. Specialists should find in the smear, scraping, taken from the wound, acantholytic cells. If they are diagnosed, the diagnosis is accurate.
- Pathohistological studies. The specialist visualizes the signs of acantholysis( stratification between the cells of the spinous layer).
Differentiation of
True pemphigus should be differentiated from a variety of diseases that occur on the mucosa inside the oral cavity:
- allergy to drugs;
- of erythema( multiforme exudative);
- pemphigoid;
- Dermatitis Dermatitis( herpetiformis);
- red lichen planus( bullous);
- non-acantholytic pemphigus( benign).
For the treatment of pemphigus in newborns, children and adults, read below.
Treatment of acantholytic pustules
If you suspect a true pemphigus, contact your dermatologist, dentist. Before the beginning of therapy, the specialist specifies the shape of the dermis lesion.
Usually treatment is medicated. You can also use the therapeutic method, some of the traditional medicine.
Therapeutic treatment
Local treatment is prescribed for the early healing of ulcers, anesthesia. Baths for the oral cavity accelerate the epithelization of the injured mucosa. In addition to the trays use:
- lubrication of mucosal corticosteroid ointments;
- applications on the mucosa;
- use of antiseptic drugs;
- sanitation of the oral cavity;
- rinsing with potassium permanganate solution( it should be warm);
- rinse with chlorhexidine( 0.02%);
- treatment of the red border of the lips with ointments with antibiotics, corticosteroids;
- rinse with chloramine( 0.25%).
- To accelerate the healing of wounds inside the oral cavity, laser therapy is used( using an infrared, helium-neon laser).
- To reduce the side effect of the use of cytostatics, glucocorticoids, it is recommended to perform hemosorption, plasmapheresis.
Medication-based
Pemphigus therapy uses corticosteroids. These drugs are considered the main weapon in the fight against dangerous pathology. Specialists usually prescribe such drugs:
- Prednisolone.
- Dexamethasone( "Dexazone").
- Methylprednisolone( Urbazone, Metipred).
- Triamcinolone( "Kenacort", "Polcortolone").
The dosage is determined by the doctor, taking into account the condition of the patient. Immunosuppressants, glucocorticoids can also be used. Many patients are helped by cytostatics( "Azathioprine", "Cyclophosphamide", "Metrotrexate").
Prevention of
After secondary therapy, secondary prevention is necessary. It is concluded in the use of hormonal means( course therapy).
It is also necessary to have an annual sanatorium treatment, which is necessary to restore the functioning of diseases of the gastrointestinal tract, the neuro-vascular system.
It is recommended that the healer avoid direct sun rays.
Complications of
Because of prolonged use of corticosteroids, patients often experience side effects:
- enhanced thrombus formation;
- increased blood pressure;
- osteoporosis of bone tissue;
- increase in glucose level in urine.
Forecast
It is quite serious even if the therapy is started in time. After taking corticosteroids for a long time you need to undergo sanatorium treatment to restore the digestive system, cardiovascular system. In most cases, due to complex rational treatment( the use of glucocorticoids is mandatory), specialists are able to change the fatal course of the disease.