How does a disease like pemphigus manifest itself? Treatment and symptoms of this disease will be discussed below. You will also learn about the causes of this pathological process and how to diagnose it.
Basic information
Pemphigus, pictured in this article, is a group of rare but very severe, potentially fatal and disabling autoimmune vesiculobullous diseases that affect the skin and mucous membranes.
Specialists identify the following types of this disease:
- pemphigus vulgaris;
- vegetative form;
- leaf-shaped;
- seborrheic (may have names such as Senier-Uscher syndrome or erythematous).
General characteristics of the disease
Pemphigus vulgaris is a category of diseases of the subcutaneous tissue and skin, which occurs in a chronic form, with frequent relapses.
This disease is hormonally dependent. It is characterized by a progressive course, as well as the formation of intraepidermal blisters.
Most often, pemphigus vulgaris affects people aged 50 and over.
Why does this disease occur? Othe reasons for the development of this disease, there are only hypotheses.
Pemphigus: the most likely causes
Before starting the treatment of the disease in question, it is very important to find out why this autoimmune disease has arisen in a particular patient. Unfortunately, finding the cause of the development of pemphigus is quite difficult. Experts have been struggling with this problem for many decades. During this time, they put forward only hypotheses:
- Exogenous factors, that is, taking medications, including penicillin and its derivatives, various interferons and others.
- Endogenous factors, including immune and genetic factors.
- Physical factors (may be affected by extensive burns and radiation received).
- Endocrine (for example, hormonal failure in the human body).
- Viruses (herpesviruses).
- Intake of certain foods.
Based on these factors that explain the likely occurrence of the disease, we conclude: pemphigus vulgaris can be immunological, endocrine, infectious, neurogenic, toxic, etc. However, it should be noted that it is still unknown whether such changes are primary or they are of a secondary nature, in response to the impact of the root cause.
Thus, the inability to identify the exact cause of the autoimmune disease in question greatly complicates its timely diagnosis, so many people very often develop severe complications.
Generation mechanism
How is it happeningthe development of a disease such as pemphigus? You can see a photo of the initial stage of this disease in this article. Experts say that the reason for the development of such a pathological process is the formation of autoaggressive antibodies to proteins belonging to the desmoglein family. The latter are a kind of "glue" that connects adjacent epidermal cells through special connecting elements called desmosomes.
After activated lymphocytes and autoantibodies attack desmogleins, the epidermal cells separate from each other, and the epidermis becomes porous and “sticky”, as a result of which it easily exfoliates and is exposed to the penetration of various fungi and bacteria. In medical practice, this phenomenon is commonly called acantholysis.
As a result of the described process, the patient has blisters on the skin, as well as in its thickness. At the same time, they are filled with exudate and constantly fester. Over time, the bubbles exfoliate from the integument, exposing the tissues and forming purulent and infected ulcers. In advanced cases, such formations can cover almost the entire surface of the human body.
Historical background
Initially, experts knew almost nothing about dermatological diseases. At the same time, the term "pemphigus" was applied to all lesions of the mucous membranes and skin, which were accompanied by acantholysis, the formation of vesicles and detachment of the integument with the development of suppurating ulcers. However, in 1964, in one of the medical journals,an article that changed the understanding of doctors about the disease in question, as well as the approach to its diagnosis and treatment. Since that time, the presence of antibodies to desmogleins in the blood plasma of patients has become the main criterion for detecting pemphigus.
By the way, another article was published in 1971, which examined in detail the autoimmune nature and mechanism of the development of this disease.
Main symptoms
Blisters on the skin, resulting from the development of vulgaris or ordinary pemphigus, are the very first sign of the development of the disease. It should be noted that this type of disease is most common. According to experts, it accounts for up to 77% of all identified forms of pemphigus.
Lesions in the form of blisters affect not only the patient's skin, but also the mucous membrane of his mouth and throat. Subsequently, they spread to the limbs, external genitalia, face, and more.
What should you know about these dermatological conditions? As a rule, pemphigus develops suddenly. At the same time, tense blisters of a small size are formed on apparently he althy skin, which become sluggish with noticeable speed. Their contents are a clear serous fluid (slightly cloudy).
After opening the papules, eroded surfaces form, which subsequently heal, but leave traces of brown pigmentation.
This autoimmune disease is characterized by severe chronic course. WhereinIt should be noted that in some people, without any treatment, there was a spontaneous improvement in the condition, which was followed by an exacerbation.
Very often, pemphigus vulgaris is accompanied by a secondary infection (candidiasis).
Due to the loss of protein, fluid and infectious consequences, the prognosis of this disease in severe forms is unfavorable.
Disease detection
How is pemphigus vulgaris detected? Diagnosis of this disease is carried out in a hospital. In this case, the presence of the disease is detected on the basis of clinical symptoms and test results.
The first include:
- Asboh-Hansen symptom. This sign is revealed by pressing a finger or a coverslip on a whole (that is, not yet opened) bubble. This procedure contributes to the exfoliation of the epidermis in the area that is adjacent to the papule, as well as an increase in its area due to the pressure of the fluid inside.
- Nikolsky's symptom. This symptom is detected in the process of capturing pieces of the bladder with tweezers and rubbing a section of apparently intact skin near the site of the lesion with a finger. In this case, detachment of the epidermis occurs.
It should be noted that the listed symptoms of pemphigus vulgaris are not specific, but diagnostic. At the same time, it must be remembered that such manifestations can occur with other diseases.
Lab tests
How is pemphigus vulgaris diagnosed? Treatment for thisautoimmune disease should be exercised only after a medical examination. To detect such a disease, use:
Histological analysis involving the examination of smears or so-called blistering impressions to identify acantholytic cells (that is, epidermal cells that have undergone morphological changes)
It should be especially noted that based on the data of histological analysis, it is impossible to draw a conclusion about the development of an autoimmune disease. This is due to the fact that there are other diseases with a similar picture.
Immunofluorescent method to detect intracellular deposits of immunoglobulin G and A, as well as to determine the main and secondary antigens - desmoglein-3 and desmoglein-1. This diagnostic method is the most accurate
Thus, the diagnosis of pemphigus vulgaris is made on the basis of the combined data of clinical symptoms and the clinical picture of the disease, as well as the results of immunofluorescent and histological examination methods.
Treatment
At the very beginning of the development of pemphigus vulgaris, the patient is prescribed glucocorticoids. The drugs of this group are taken in loading doses. The appointment of such a drug in large quantities has vital indications. As for contraindications and side effects, they are secondary.
What glucocorticoids are prescribed for the disease under consideration? Drugs for the treatment of vulgar vesicles can be as follows: Prednisolone, Dexamethasone and Triampcinolone.
After the improvement of the patient's condition, thenis in the absence of new blisters, the dose of drugs is gradually reduced and switched to maintenance in order to prevent relapse. Such treatment of patients is quite lengthy.
In addition to glucocorticoids, patients may be prescribed cytostatic immunosuppressants, including Methotrexate, Azathioprine, or Prospidin. They are necessary to relieve the adverse reactions that have arisen after taking essential medicines.
Other Therapies
If there are indications, then in case of autoimmune diseases, the patient can be prescribed antibiotics, as well as drugs that support the cardiovascular system, regulate blood pressure and normalize the liver and kidneys.
It is also mandatory to take potassium-containing products, calcium and vitamins. For external treatment of pemphigus, anti-inflammatory drugs, herbal tinctures and decoctions can be used.
It should also be noted that depending on the patient's condition, procedures such as haemasorption, blood transfusion and plasmapheresis may be prescribed.
