What is pemphigus vulgaris?

Pemphigus vulgaris is one of the rare, recurrent autoimmune diseases that causes blistering of the skin and mucous membranes (for example, the mouth, nose, throat, and genitals).

Related article: What are autoimmune diseases?

In pemphigus disorders, the immune system makes a mistake, seeing cells in the skin and mucous membranes as foreign, and an immune response is triggered.

Affected individuals have high concentrations of antibodies produced by the immune system. These bind to a specific protein in the skin (desmoglein 3 and sometimes desmoglein 1) and cause interference with the normal function of the skin. The cells no longer stick together and are separated from each other.

This causes the burn-like lesions or blisters typical of pemphigus vulgaris. Symptoms usually appear in the mouth first, but lesions can cover a significant area of ​​skin. The disease is not contagious.

Although desmoglein antibodies are known to cause pemphigus vulgaris, it is not known why people develop these autoantibodies in the first place. Genetic factors are believed to be important, but there may be other factors that help trigger the disease.

Who is it seen in?

Pemphigus vulgaris most often develops between the ages of 30 and 60. However, the disease can also occur in children and the elderly. Men and women are affected with equal frequency. In general, this condition is a very rare disease. Only one to five people out of a million have this condition.

What causes pemphigus vulgaris?

Pemphigus vulgaris is an autoimmune disease, which means that the disease process relies on a misguided immune system response. People with pemphigus vulgaris make antibodies (autoantibodies) that are incorrectly directed against the body’s own tissues or proteins called desmogleins.

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These desmogleins provide cell-cell connection between the two layers of the epidermis and this holds the skin together. Pemphigus autoantibodies destroy this link.

Because of this, large blisters form on the skin, similar to burns. An inflammatory reaction loosens and kills the upper layers of the skin. There are wide open areas on the skin and mucous membranes that heal poorly and are easily infected.

The exact causes of the disease are still unclear. However, several studies suggest that an inherited tendency is at least involved. Some patients also suffer from another autoimmune disease.

Pemphigus vulgaris sometimes occurs as an accompanying severe cancer symptom. Possible triggers of the disease can be burns and certain medications, as well as autoantibodies.

What are the symptoms of pemphigus vulgaris?

Blisters often develop in the mouth before affecting the skin after a few weeks or months. There may be periods when the blisters are severe (flares), followed by periods when they heal and fade (remission). It is impossible to predict when this might happen and how severe the flare-ups will be.

Blisters in the mouth often develop into painful sores, which can make it very difficult to brush teeth, eat and drink. Hoarseness may occur if the bubbles spread to the voice box (larynx) .

Sores on the skin can coalesce to spread over large, painful areas of skin before crusting and forming crusts. Although the affected skin is sometimes permanently discolored, they usually do not leave any scars.

Besides having blisters in the mouth, they can also develop in other areas such as the nose, throat, anus, genitals, and vagina. The thin membrane that covers the front of the eye and the inside of the eyelids (conjunctiva) may also be affected.

How is pemphigus vulgaris diagnosed?

Due to the rarity of the disease, erosions in the oral mucosa area are not usually considered the first manifestations of pemphigus vulgaris. Skin symptoms are also easily confused with other autoimmune diseases in which the skin is puffy.

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If pemphigus vulgaris is suspected, the doctor will take a tissue sample (biopsy) from the side of the bladder for microscopic examination under local anesthesia. Under the microscope, one can see the skin cells segregating from each other in the way typical of the disease.

Desmoglein antibodies can be made directly visible in biopsy with a special staining method. Autoantibodies against Desmoglein 1 and Desmoglein 3 protein molecules can also be detected in the blood of most of those affected. Regular control is helpful during the course of the disease. The number of these antibodies is directly related to the disease activity present.

How is pemphigus vulgaris treated?

The cause of pemphigus vulgaris cannot be cured. If the triggering factors are known, for example some drugs, it is recommended to stop taking these drugs. It is also recommended to protect the skin from the sun’s rays with appropriate clothing.

local skin treatment

Applying antiseptic-containing creams or ointments and applying antiseptic compresses reduce the risk of bacterial infections. There are eye drops with antiseptic for the eyes. Local treatment with cortisone preparations, such as ointments or mouthwashes, promotes healing.

Internal treatments

Medication is to suppress the overreaction of the body’s own defenses, thereby preventing blistering as much as possible.

  • Cortisone preparations in combination with immunosuppressants: Cortisone preparations in combination with other immunosuppressants (immunosuppressants) act as essential medicine. The combination of the two active principles increases the effectiveness of individual substances without having to dose a single drug excessively high.
  • Immunoglobulins: If treatment with cortisone and immunosuppressive agents is not successful, immunoglobulins may be used. Immunoglobulins (antibodies) are proteins that are important to the body’s immune system. The doctor injects immunoglobulins intravenously to treat pemphigus vulgaris. Patients are often given additional drugs that suppress the immune system.
  • Immunoadsorption: Immunoadsorption is another treatment method used primarily in very severe cases. In this method, a machine filters disease-causing autoantibodies from the patient’s blood. This treatment is usually carried out in combination with immunosuppressive drugs.
  • Rituximab: This is a monoclonal antibody that targets a specific protein on the surface of lymphocytes. It can improve symptoms in patients with pemphigus vulgaris and is mainly used in patients who have had an inadequate response to other treatments. Treatment is by infusion.
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Remember, your doctor will decide which medicine to take.

Other precautions

In addition, patients should be very careful with their sensitive skin. It is recommended that you avoid severe skin stress, for example avoid stressful situations caused by very tight clothing or sports involving physical contact. It is important to eat a balanced and adequate diet with changes in the mucous membrane in the mouth area.

Chances of recovery and outlook

Pemphigus vulgaris is a serious illness that, if left untreated, is almost always fatal within one to three years. A causal cure is still not possible, but immunosuppressants and other new treatments have significantly reduced the risk of the disease and greatly improved the quality of life of those affected.

Unfortunately, some of these drugs have significant side effects. Permanent treatment with cortisone can cause diabetes and osteoporosis and impair kidney function. A major problem with immunosuppressive therapy is the increased risk of infection.

In addition to starting treatment as early as possible, constant close cooperation with the doctor is essential for the success of the treatment. The doctor adjusts the dose of the drug most appropriately to the current disease activity. Regular laboratory tests help quickly discover possible side effects and prevent permanent damage.

The prognosis of pemphigus vulgaris disease depends on the degree of skin damage and hence the required dosage of immunosuppressive agents. As studies have shown, the first five years of illness are particularly critical, after which the prognosis improves and many patients enjoy a high quality of life despite serious illness.

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