Pemphigus vulgaris

definition

The word pemphigus comes from the Greek and means bubble. Colloquially, pemphigus vulgaris is also known as bladder addiction. The disease pemphigus vulgaris is one of the blistering diseases. In this context, pemphigus vulgaris belongs to the pemphigus group. This means it is a chronic skin disease that is characterized by blistering of the skin and mucous membranes.

This rare disease affects women and men equally often. About only 1-5 out of 1 million people develop pemphigus vulgaris. However, the disease occurs differently in different regions. In Central and Eastern Europe, for example, the disease occurs much more often than in other regions.

Causes of Pemphigus Vulgaris

Pemphigus vulgaris has an immunological cause. It is an autoimmune disease. This means that instead of fighting for the body, the body's defense system fights against the body. The reason for this are dysregulated immune system processes.

The "pemphigus autoantibodies" are directed against Desmoglein 3. Desmogelins are proteins in our body that ensure that connections between cells can develop. When this mechanism is disrupted, the characteristic blisters of pemphigus vulgaris can develop.

In the course of inflammation, the upper layers of the skin eventually become detached and die off. However, it has not yet been fully clarified why autoantibodies are directed against this protein and how they work. There are two guesses. On the one hand, it is assumed that the autoantibodies impair the connection between the desmogelins. On the other hand, it is assumed that the autoantibodies initiate the cell death of the skin cells.

Pemphigus vulgaris is also associated with other autoimmune diseases, such as some form of anemia (pernicious anemia), Cancers and diseases that cause muscle weakness (Myasthenia) cause.

If there is a genetic predisposition for these diseases, pemphigus vulgaris can also be triggered by viruses, various drugs, burns, and UV or X-ray radiation. According to the National Institutes of Health, possible drug triggers include pencillamine and ACE inhibitors. Various studies have discussed whether pemphigus vulgaris is generally related to a genetic predisposition. This could possibly explain the regional differences.

Diagnosis of pemphigus vulgaris

At the beginning of every diagnosis there is an interview with the patient. This is also known as anamnesis. The doctor will also look at the affected parts of the body. Blisters on the oral mucosa, in other parts of the body and a positive Nikolski sign can indicate pemphigus vulgaris.

The Nikolski sign is checked to determine the tendency to form blisters. The doctor tests how the bubbles behave when the pressure is pushed.

Furthermore, the bubbles or their contents can be viewed microscopically. For this purpose, a tissue sample is taken under local anesthesia. If the doctor sees rounded skin cells under a microscope, then the Tzanck test is positive. This means that the doctor sees a typical change in the layers of the skin under the microscope.

Proof of "Pemphigus antibodies“Can confirm the suspected diagnosis. These can be examined in a number of ways. One possibility is to display them using a special coloring method. The number of antibodies correlates with the severity of the disease. In the advanced stage, the inflammation parameters in the blood count, as well as the electrolytes and serum proteins are examined more closely. Regular check-ups should be carried out as the disease progresses.

Concomitant symptoms

The first symptoms of pemphigus vulgaris are in most cases not very pronounced over a long period of time. As a result, they are often not recognized.

Patients have blistering in one or more parts of their body. These blisters are often flaccid and fragile. You are on less inflamed skin. They are usually filled with a transparent liquid. After a short time these burst open.

This can result in erosions, crusts, scars and hyperpigmentation. Often the mucous membrane of the mouth is affected and can be or remain the sole site of complaints for a long time. The parts of the body where the symptoms occur particularly frequently are the scalp, the oral mucosa, mechanically stressed skin areas and the face. These sites are also known as predilection sites.

Only in the case of extensive infestation, in addition to blistering, loss of appetite, fatigue, a feeling of illness and fever occur.

On the mucous membrane

Pemphigus vulgaris often manifests itself on the lining of the mouth. In more than 50%, the disease begins in this area. Whitish deposits and abrasions are typical. The abrasions are also called in technical jargon Erosions designated. As a rule, the blisters on the mucous membranes burst more quickly than on other parts of the body. The sometimes bleeding abrasions are often very painful for the patient. If pemphigus vulgaris has manifested itself on the mucous membrane, a balanced and adequate diet should be ensured.

In addition to the oral mucosa, the genital mucous membranes can also be affected.

Is Pemphigus Vulgaris Contagious?

Superinfection can develop as part of pemphigus vulgaris. This is contagious, while pemphigus vulgaris is not itself contagious. That said, pemphigus vulgaris cannot be transmitted from person to person.

One suspects, however, a hereditary predisposition to be part of the cause. If family members have or have suffered from pemphigus vulgaris, the offspring are more likely to develop it as well.

In principle, all people of all ethnicities, ages and genders can contract pemphigus vulgaris. However, it was found that the disease occurs in certain groups of people in addition to or in addition to the familial accumulation. This affects people of Mediterranean descent, people who live in the Brazilian rainforest, Eastern European Jews, and people of middle or older age.

This is how pemphigus vulgaris is treated

For the treatment of pemphigus vulgaris, a distinction is made between external, local and internal, systemic therapy.

The external, local therapy serves to alleviate the symptoms. It does not treat the cause of the disease. The disease cannot be stopped with symptomatic treatment. Different preparations are used depending on which area of ​​the body is affected. Various antiseptic or partially cortisone-containing ointments, eye drops and mouthwashes are used for local, external treatment.

The aim of internal, systemic therapy is to suppress the excessive reactions of the immune system. Be for this Glucocorticoids utilized. In the case of acute complaints, maximum doses are only selected temporarily. In the case of long-term treatment, attempts are made to keep the dosage as low as possible in order to limit side effects. In addition, immunosuppressants such as Azathioprine, used. In the case of advanced or severe pemphigus vulgaris Cyclophosphamide, Cyclosporine A and Methotrexate used.
If cortisone preparations and immunosuppressants do not work, immunoglobulins are given. These are antibodies that influence certain processes in the body. Since these are proteins, the antibodies cannot be taken orally in tablet form, but must be injected into the vein.
Another treatment that is not given in tablet form but rather via an infusion is treatment with Biologicals. In particular Rituximab used when all other measures are ineffective.
In some cases, immunoadsorption or plasmapheresis is advisable. Here, the disease-causing autoantibodies are filtered out of the patient's blood. This is done using a special machine. Plasmapheresis is not as effective as immunoadsorption and has therefore lost its importance. Several medications and measures are often combined with one another.

In addition, the basis of the treatment of pemphigus vulgaris forms the instructions for careful handling of the skin. Patients should take care of their skin accordingly and avoid excessive skin exposure. For example, clothing that is too tight should not be worn, exposure to strong sunlight should be avoided and sports that involve skin contact should be avoided.

Is it curable?

The prognosis has since improved. Since the cause is not yet fully understood, a complete cure is not possible. But with certain drugs and measures, the destructive processes in the body can be suppressed. This can slow down and alleviate the course of the disease. The lifespan and quality of the patients could thus be increased many times over. Nevertheless, 5-10% of patients worldwide still die as a result of the disease.

When will I be healthy again?

Pemphigus vulgaris is a chronic skin condition that occurs in intermittent phases. This means that there are phases when the symptoms are more severe and phases when the symptoms are less pronounced. But the disease itself persists due to its chronic course.

Some authors divide the disease into two stages.
Accordingly, the first stage lasts Initial phase, about up to a year.
The next stage will Generalization phase called, which can appear again and again in batches.

Overall, the disease can have a different duration. If left untreated, chronic pemphigus vulgaris was fatal in most cases after 1-3 years. The duration of the illness depends on the severity of the skin damage. Studies have shown that the first 5 years of illness are particularly severe. After that, the prognosis, life span and quality of life can improve.