Definition
The word pemphigus comes from the Greek and means bubble. Colloquially, pemphigus vulgaris is also called bladder addiction. The disease pemphigus vulgaris is one of the bladder forming diseases.
Pemphigus vulgaris belongs in this context to the pemphigus group. This means it is a chronic skin disease characterized by blistering of the skin and mucous membranes. Women and men are equally affected by this rare disease.
Only about 1-5 out of 1 million people suffer from pemphigus vulgaris. However, the disease occurs with varying frequency in different regions. In Central and Eastern Europe, for example, the disease occurs much more frequently than in other regions.
Causes of pemphigus vulgaris
Pemphigus vulgaris has an immunological cause. It is an autoimmune disease. This means that the body’s own defense system fights against it instead of fighting for the body.
The reason for this are misregulated processes of the immune system. The “pemphigus autoantibodies” are directed against desmoglein 3. Desmogelins are proteins in our body that ensure that connections between cells can be made.
If this mechanism is disrupted, the characteristic blisters of pemphigus vulgaris can develop. In the course of inflammatory processes, they eventually detach and die off the upper layers of the skin. However, it is not yet fully understood why autoantibodies are directed against this protein and how they act.
There are two speculations. On the one hand, it is assumed that the autoantibodies interfere with the connection of the desmogelins with each other. On the other hand, it is assumed that the autoantibodies initiate the cell death of the skin cells.
In addition, pemphigus vulgaris is associated with other autoimmune diseases, such as a form of anaemia (pernicious anaemia), cancer and diseases causing muscle weakness (myasthenia). 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. In various studies it is discussed whether pemphigus vulgaris is generally related to a genetic predisposition. This could possibly explain the regional differences.
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