How does a disease like pemphigus manifest? The treatment and symptoms of this disease will be discussed below. You will also learn about the reasons for the development of this pathological process and how to diagnose it.
Basic information
Pemphigus, the photo of which is presented in this article, is a group of rare, but very serious, potentially fatal and disabling vesiculobullous autoimmune diseases that affect the skin and mucous membranes.
Specialists distinguish the following types of this disease:
- pemphigus vulgaris;
- vegetative form;
- leaf-shaped;
- seborrheic (may have names such as Senir-Asher syndrome, or erythematous).
General characteristics of the disease
Pemphigus vulgaris is a category of diseases of the subcutaneous tissue and skin, which proceeds in a chronic form, with frequent relapses.
This disease is hormone dependent. It is characterized by a progressive course, as well as the formation of intraepidermal blisters.
Most often, pemphigus vulgaris affects people aged 50 or more.
Why does this disease occur? There are only hypotheses about the causes of this disease.
Pemphigus: the most likely causes
Before starting treatment of the disease in question, it is very important to find out why this autoimmune disease occurred in a particular patient. Unfortunately, finding the cause of pemphigus development 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 other things.
- Endogenous factors, including immune and genetic.
- Physical factors (extensive burns and radiation may affect).
- Endocrine (for example, hormonal failure in the human body).
- Viruses (herpes viruses).
- The intake of certain foods.
Based on these factors explaining 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 not known whether such changes are primary or whether they are secondary nature, in response to the impact of the root cause.
Thus, the inability to identify the exact cause of the occurrence of the autoimmune disease in question significantly complicates its timely diagnosis, so many people very often develop severe complications.
Nucleation mechanism
How does a disease like pemphigus develop? 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, epidermal cells separate from each other, and the epidermis becomes porous and “sticky”, as a result of which it easily peels off and undergoes the penetration of various fungi and bacteria. In medical practice, this phenomenon is called acantolysis.
As a result of the described process, the patient develops blisters on the skin, as well as in its thickness. Moreover, they are filled with exudate and constantly fester. Over time, the vesicles 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.
History reference
Initially, experts did not know anything about dermatological diseases. In this case, the term “pemphigus” was applied to all lesions of the mucous membranes and skin, which were accompanied by acantholysis, the formation of vesicles, and the detachment of integuments with the development of suppurating ulcers. However, in 1964, an article was published in one of the medical journals that changed the doctors' understanding of the disease in question, as well as the approach to its diagnosis and treatment. Since that time, the presence in the blood plasma of patients of antibodies to desmogleins has become the main criterion for the detection of pemphigus.
By the way, in 1971 another article was published in which the autoimmune nature and mechanism of the development of this disease were studied in detail.
Main symptoms
Bubbles on the skin resulting from the development of vulgar or ordinary pemphigus are the very first sign of the development of the disease. It should be especially 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 skin of the patient, but also the mucous membrane of his oral cavity and pharynx. Subsequently, they spread to the limbs, external genitalia, face, and more.
What you should know about such dermatological diseases? As a rule, pemphigus develops suddenly. At the same time, tense blisters of a small size are formed on the apparently healthy skin, which, with appreciable speed, become sluggish. Their contents are a clear serous fluid (slightly cloudy).
After opening the papules, erosive surfaces form, which subsequently heal, but leave traces of brown pigmentation.
Such an autoimmune disease is characterized by a severe course in a chronic form. It should be noted that in some people without any treatment there was a spontaneous improvement in the condition, 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. Moreover, the presence of the disease is detected on the basis of clinical symptoms and test results.
The first include:
- Symptom of Asbo-Hansen. This symptom is detected by pressing a whole bubble (that is, not yet opened) with a finger or coverslip. This procedure contributes to the exfoliation of the epidermis in the area that is adjacent to the papule, as well as increasing its area due to the pressure of the liquid inside.
- Symptom of Nikolsky. This symptom is detected in the process of capturing pieces of the bladder with tweezers and rubbing with a finger a portion of externally intact skin near the lesion. In this case, exfoliation of the epidermis occurs.
It should be noted that the listed symptoms of pemphigus vulgaris are not specific, but diagnostic. It should be remembered that such manifestations can occur with other diseases.
Laboratory tests
How is pemphigus vulgaris diagnosed? The treatment of this autoimmune disease should be carried out only after a medical examination. To identify such a disease, apply:
- Histological analysis, which involves the study of smears or so-called fingerprints of blisters to identify acantholytic cells (i.e. epidermal cells that have undergone morphological changes).
It should be especially noted that based on the data of histological analysis, a conclusion about the development of autoimmune disease cannot be made. This is due to the fact that there are other diseases with a similar picture.
- Immunofluorescence method, which allows 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 “vulgar pemphigus” 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 the immunofluorescence and histological examination methods.
Treatment
At the very beginning of the development of vulgar pemphigus, glucocorticoids are prescribed to the patient. Drugs in this group are taken in shock doses. The purpose of such a drug in large quantities has vital indications. As for contraindications and side effects, they are secondary.
What kind of glucocorticoids are prescribed for the disease in question? Preparations for the treatment of pemphigus vulgaris can be as follows: Prednisolone, Dexamethasone and Triampcinolone.
After the patient's condition improves, that is, in the absence of new blisters, the dose of drugs is gradually reduced and switched to a maintenance one in order to prevent relapse. Such treatment of patients is quite lengthy.
In addition to glucocorticoids, patients can be prescribed cytostatics – immunosuppressants, including Methotrexate, Azathioprine, or Prospidine. They are necessary to relieve the occurring adverse reactions after taking essential drugs.
Other therapies
If there are indications, then with autoimmune diseases, the patient can be prescribed antibiotics, as well as drugs that support the work of 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, such procedures as hemabsorption, blood transfusion and plasmapheresis may be prescribed.