Pemphigus vulgaris
This group of diseases of the body be directed defense against itself (autoimmune reaction). attacked with the mechanisms that are otherwise the distance exogenous substances (for instance, serve pathogens), which now own cells. Here proteins are formed: the antibodies. These antibodies recognize certain cells and lead to their destruction.
In pemphigus vulgaris, the autoimmune reaction is directed against cell structures (Desmoglein III on keratinocytes) in the outermost skin layer (epidermis). The normally strong bond between the cells in the epidermis is achieved (acantholysis). It is easy to tear. It can form bubbles (Pemphix: Greek bladder).
Causes of this disease are still unknown. A predisposition to disease pemphigus vulgaris, is probably inherited.
Frequency
The age of onset is between the ages of 40 and 70 Year of life. The disease is rare: about 1-5 people a year fall ill in 1000 000.
Symptoms
- Groovy bubble remains in the mouth
- The skin can be removed by lightly rubbing (so-called direct Nikolsky's sign)
- Later followed by formation of flaccid vesicles
- By bursting of bubbles or the shedding of the epidermis caused by moving bright red skin wounds, the wet, bleed easily and burn
Complications
- The skin wounds may be infected by bacteria. In severe cases it can result leading to blood poisoning.
- For infestation of the mouth area, the food intake may be impaired. Consequences are liquid, protein, electrolyte depletion (salt wasting) and malnutrition.
- During pregnancy, antibodies can be transferred to the unborn baby, so sick of this.
Other forms
Other forms of pemphigus vulgaris occurring (P. vegetans, P. herpetiformis, P. foliaceus, paraneoplastic pemphigus, drug-induced pemphigus) differ mainly by the affected body region and / or by its cause.
Therapy
- Administration of cortisone to inhibit the production of antibodies (eg, prednisolone)
- Addition of other drugs such as immune-damping azathioprine, mycophenolate mofetil, cyclophosphamide, chlorambucil, cyclosporin A
- Hemodialysis (plasmapheresis) to remove the antibodies from the blood
- Administration of so-called immunoglobulins