Leaf-shaped( exfoliative) pemphigus: symptoms, causes, treatment, prognosis

August 22, 2017 23:00 | Pemphigus

Skin diseases are considered very popular problems.These pathologies are characterized by peculiar manifestations.One of the problems of the dermis is the leaf-shaped pemphigus.Many experts consider this disease a consequence of the failures noted in the work of the immune system.

Features of the disease

Pemphigus has many subspecies.The leaf form of the disease has such a specific name due to the fact that multi-layer crusts appear on the dermis.Their appearance is similar to stacks of sheet material.These layered crusts appear on top of the erosions that formed after the bubbles.

This lesion of the dermis is the most common form of pemphigus, which dermatologists note in children.As for adult patients, this pathology prevails in frequency over the vegetative type of pemphigus.

This lesion of the dermis is characterized by an autoimmune development mechanism, with the appearance of autoantibodies to skin cells. The disease is very dangerous( possibly fatal) due to cachexia, the att

achment of a secondary infection.

So far, scientists have not established the reasons that trigger changes in the immune system.There are versions of the effect on the body of exogenous factors( if a patient has a genetic predisposition).

Symptoms of foliar pustules

Initially, the skin of the patient appears areas of skin with a specific redness, which is covered with blisters( flattened, flabby).Sometimes the disease can develop like vulgar pemphigus, Dühring's dermatitis, and other types of dermatitis.In some cases, the blisters are very weakly expressed.

The crust formed in the formed bubbles is very thin, so they break very quickly.After the bubble bursts, the erosion of bright red color remains in its place.When a rupture follows a lot of detachable, it withers, forming crusts.Many layers of this crust appear after separation from the wound of new portions of the separated.This results in layered crusts, because of which the disease is called leaf-like pemphigus.

The healing of erosions is rather slow.The formation of new bubbles occurs even under the crusts.This process is practically not interrupted.If the disease progresses, the nearby bubbles merge.So the defeat captures very extensive areas of the dermis.

As for the general condition of the patient, in the beginning it is quite normal.Deterioration is noted after an increase in the affected area.The patient is concerned:

  • pain, felt even with a slight movement;
  • worsening of sleep;
  • decreased appetite;
  • temperature increase;
  • weight reduction;
  • manifestation of mental disorders;
  • hair loss( with infection of the dermis of the head);
  • thickening, tarnishing of nails;
  • abaissement of nail plates( in rare cases);
  • development of cachexia.

Diagnosis of such a disease as foliar pemphigus in children and adults is described below.

Diagnostics

General

  1. External inspection .Most often, experienced dermatologists make an accurate diagnosis of the patient's symptoms, which we painted above.
  2. Smear-print .It is necessary for the detection of acantholytic cells, which are characteristic of all forms of pemphigus.
  3. Histological examination of .Thanks to him, a specialist can detect between the layers of the epidermis crevices, blisters, degeneration of cells of the granular layer.
  4. Immunological studies of .They are needed to confirm the autoimmune mechanism of the disease.Direct / indirect REEFs are not always effective, they provide an opportunity to distinguish between a vulgar, leafy type of pathology.

Differential

In addition to these studies, differential diagnosis of the affected dermis from:

  • of lupus erythematosus;
  • of erythroderma;
  • of Duhring's dermatitis;
  • seborrheic eczema;
  • of Lyell's Syndrome.

Next we will tell you about the treatment of foliar pemphigus.

Treatment of

This disease needs to be treated in a timely manner.Therapy should be conducted under the supervision of a dermatologist after an accurate diagnosis.Treatment is carried out exclusively medicamentally.

Therapeutic way

As an adjunct to medical treatment, it is possible to use a therapeutic method.Dermatologists can advise local baths, which contain disinfectants, anti-inflammatory agents, astringents.So, you can use a decoction broth prepared from the bark of oak.

Medication

Treatment is aimed at reducing the production of antibodies by the body to its own tissues.Essential medicines used in the therapy of this dangerous disease are corticosteroid hormones.

Most often dermatologists prefer prednisolone.The dermatologist selects the treatment regimen on an individual basis.To start a dose is chosen, which is equal to 80 - 100 mg of the drug per day.It is necessary 2/3 of the prescribed daily dose to use the sutra.

A noticeable effect of the hormone therapy that is being introduced appears on the 10th to the 14th day of the use of the drug.From this time, you should reduce the dose of the drug used.The first reduction is usually 25 - 30%, after which the dosage reduction is introduced more smoothly.

Glucocorticosteroids can be assigned:

  • Triamcinolone.
  • "Prednisolone".
  • "Dexamethasone".
  • "Methylprednisolone".

If the case is very severe, the dermatologist prescribes immunosuppressive agents:

  • "Methotrexate".
  • "Azathioprine".

The effect in the treatment of the disease was noted with the simultaneous use of corticosteroids, synthetic antimalarials( "Hydroxychloroquine", "Chloroquine").

Folk way

Different variants of folk medicine can be used as an adjunct to the therapy that the dermatologist prescribed.To treat affected areas, you can use:

  • mushroom-raincoat( its flesh);
  • leaves of nettle( juice squeezed out of them);
  • is an oily tincture made from fresh walnut leaves.

Prevention of the disease

The essence of prevention is the maintenance of a healthy lifestyle.The patient is recommended:

  • high-grade food;
  • healthy sleep;
  • walks in the fresh air( the sun is recommended to be avoided).

Complications

Corticosteroid drugs after prolonged use provoke the occurrence of many complications:

  • hypertension;
  • Osteoporosis;
  • steroid diabetes;
  • gain in weight;
  • erosive diseases of the digestive tract;
  • mental disorders( acute psychosis, depression);
  • systemic angiopathy.This pathology can cause the occurrence of myocardial infarction, stroke, hemorrhagic pancreatitis;
  • thromboses;
  • Attachment of infection.

Forecast

For this disease, the dermis is very unfavorable.The disease can last very long. Most of the cases are fatal due to the infection, cachexia, dangerous complications that arise after the constant use of glucocorticoids.

Pamphlet is described in this video: