Pemphigus

General description of the disease

This is a chronic pathology of autoimmune origin that affects the skin and mucous membranes. Pemphigus can develop at any age, however, it most often affects men and women who have crossed the 40-year-old milestone, the disease is most severe in 40-45-year-old people, and is rare in children. The share of pemphigus accounts for about 1% of dermatological diseases.

Causes

The etiology of pemphigus could not be established for long enough, but studies have confirmed that the cause of this skin pathology is a malfunction of the immune system.^[3].

The job of the immune system is to defend against foreign organisms. Autoimmune diseases occur when, as a result of dysfunction, the immune system attacks the cells of the body, in the case of pemphigus, the skin. The antibodies produced by the immune system mistakenly attack the proteins in the upper layers of healthy skin. Demosomes, which are the connecting link between the cells of the skin under the attack of autoantibodies, lose their connections and are destroyed, and the vacant cavity is filled with intercellular fluid, as a result of which acantholytic vesicles are formed (hence the name of the disease).

Risk factors for the development of pemphigus can be both exogenous (infectious diseases, viruses, professional activity) and endogenous causes, including genetic predisposition. The reasons for the development of pemphigus can be a strong nervous shock, as well as pathology of the adrenal cortex.

Agricultural workers, who are often in contact with herbicides and insecticides, as well as workers in the metal industry and printing houses, are more likely to develop pemphigus.

Types and symptoms

The characteristic features of the presented pathology are small vesicles with serous contents, which are localized on the patient’s body, depending on the type of pemphigus:

• vulgar – differs in the appearance of bubbles with a thin and flaccid tire throughout the body. With a vulgar or ordinary form, bubbles at the beginning of the development of the disease are localized on the mucous membranes of the nose and mouth, so patients go to the dentist and are treated unsuccessfully, wasting time. Patients are worried about bad breath, pain in the mouth while eating, talking and swallowing saliva. Patients do not always notice tiny bubbles that are prone to spontaneous opening, so the main complaints are painful erosion in the mouth, which dentists often diagnose as stomatitis. With pemphigus vulgaris, ulcers that form when the vesicles are opened merge and form extensive lesions. Unlike stomatitis, which is characterized by erosion with a whitish coating, pemphigus ulcers have a bright pink color and a glossy surface. When the larynx is affected by pemphigus, the patient’s voice becomes hoarse;
• erythematous the form of pemphigus is characterized by the fact that it primarily affects the skin of the chest, face, neck and scalp. Rashes of a seborrheic nature with clear boundaries are covered with brown or yellow crusts; when opened, erosion is exposed. This type of pemphigus is not easy to diagnose, so the erythematous form may be localized for more than one year, and in case of an exacerbation, it may show symptoms of a vulgar one;
• leaf-shaped – rashes of an erythema-squamous nature can occur on previously affected areas of the skin, then bubbles with thin walls open up, forming erosion, which dry up and become covered with lamellar crusts. This form of pemphigus, as a rule, affects the skin, small bubbles quickly spread over healthy skin, in some cases, mucous membranes may be damaged;
• vegetative the form is manifested by bubbles in the area of ​​skin folds, in place of the bubbles, erosion with a putrid odor and purulent plaque forms over time.

In addition to rashes on the skin and mucous membranes, patients with pemphigus have general symptoms:

1. 1 fatigue;
2. 2 decreased or loss of appetite;
3. 3 weight loss even with increased nutrition;
4. 4 drowsiness.

Complications

With untimely or incorrect therapy, the bubbles spread throughout the body, merge and form large lesions. Running pemphigus is a serious danger along with a skin burn. Skin lesions affect the patient’s quality of life, the patient cannot move normally. When erosions are infected, the most common complication is pyoderma.^[4]… It is also possible the spread of inflammatory processes to internal organs, as a result of which phlegmon and pneumonia develop.

On the part of ENT, hearing loss may develop as a complication of pemphigus; mycoses prevail among dermatological complications. Complications of the cardiovascular system are manifested in the form of ischemia, angina pectoris and microangiopathy.

The risk of death in patients with pemphigus is quite high – up to 15% of patients die within 5 years after the onset of the disease.

Prevention

As a preventive measure to prevent the development of pemphigus, you should:

• change bed linen regularly;
• change underwear daily;
• timely treat skin pathologies;
• to remove from work people with pustular eruptions;
• systematic control of a dermatologist;
• limit the intake of salt, fat and carbohydrates;
• monitor blood glucose and blood pressure readings;
• strictly observe the rules of personal hygiene.

Treatment in mainstream medicine

Pemphigus treatment is long and difficult. Pemphigus suggests complex therapy:

1. 1 systemic treatment;
2. 2 local therapy;
3. 3 extracorporeal techniques.

Local therapy involves the treatment of the affected skin with healing and hormonal ointments and irrigation of erosions with painkillers.

Extracorporeal treatment involves the use of hemodialysis and plasmaphoresis.

The mainstay of pemphigus treatment is hormone therapy. The patient is prescribed pills, and hospitalized patients are given intravenous corticosteroids. The treatment regimen should be strictly adhered to, since taking hormonal drugs can cause serious side effects:

• depression;
• sleep disorders;
• hypertension;
• obesity, even with a low-calorie diet;
• steroid-type diabetes;
• excessive excitement of the nervous system;
• stool disorders.

With an exacerbation, drugs are shown that suppress the immune system. Patients with severe pemphigus may need plasma replacement. In severe forms of pathology, intravenous immunoglobulin is prescribed.

To prevent infection after opening the blisters, antibiotics are prescribed for patients with pemphigus. Dressings soaked in petroleum jelly are applied to ulcers and oozing areas. In case of exacerbation, it is recommended to wear loose clothing made from natural fabrics.

Attention!

The administration is not responsible for any attempt to use the information provided, and does not guarantee that it will not harm you personally. The materials cannot be used to prescribe treatment and make a diagnosis. Always consult your specialist doctor!