True pemphigus( acantholytic) or pemphigus: symptoms, causes, treatment, prognosis

August 22, 2017 23:00 | Pemphigus

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 as a consequence of the 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)

classification

The 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 has not been 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

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 us consider the signs peculiar to each of the forms of the true pemphigus.

Vulgar

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.

Typical signs of this form of pathology are:

  • hypersalivation;
  • pain when swallowing;
  • appearance of a specific odor in the mouth;
  • pain when talking.

After 3 to 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 derma, 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 epithelium layer is noted).

Sheet

Thin-walled bubbles appear continuously, they appear at the same place.After bursting, the bladder leaves the wounds, they form crusts.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 sifting 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 large 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 a satisfactory state.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, pustules are marked on the periphery.Near the focus of infection will be a positive symptom of Nikolsky.

Diagnostics

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 accurate.
  • Pathohistological studies.The specialist visualizes the signs of acantholysis( stratification between the cells of the thorny layer).

Differentiation

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, please contact your dermatologist, dentist.Before the start of therapy, the specialist specifies the form of the dermis lesion.

Usually, the treatment is medicated.You can also use a therapeutic method, some tools from traditional medicine.

Therapeutic method

Local treatment is prescribed for the prompt 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;
  • rinsing with chloramine( 0.25%).
  • To accelerate the healing of wounds within 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.

By medicamentous route

In the treatment of pemphigus, corticosteroids are used.It is these medicines that are considered the main weapon in the fight against dangerous pathology.Specialists usually prescribe such drugs:

  • Prednisolone.
  • Dexamethasone( "Dexazone").
  • Methylprednisolone( "Urbazon", "Metipred").
  • Triamcinolone( "Kenacort", "Polcortolone").

The dosage is set 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 disease

After the therapy, secondary prevention is necessary.It is concluded in the use of hormonal drugs( course therapy).

Also necessary is an annual spa treatment, which is necessary to restore the functioning of diseases of the gastrointestinal tract, the nervous system.

It is recommended that the healer avoid direct sun rays.

Complications

Because of prolonged use of corticosteroids, the side effects of

  • are often manifested in patients who have severe thrombosis;
  • increase in blood pressure;
  • osteoporosis of bone tissue;
  • increase in glucose level in urine.

Forecast

It's pretty serious even if you start therapy 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.