Pemphigus vulgaris

The illness Pemphigus vulgaris belongs to the category of dermatology due to its appearance. The optically visible manifestations that occur in pemphigus vulgaris are limited exclusively to skin tissue.

What is pemphigus vulgaris?

As part of the definition of the Pemphigus vulgaris we speak of a so-called autoimmune disease of the skin, which is primarily associated with the development of blisters. A causal connection between pemphigus vulgaris and autoimmune diseases of other organs as well as carcinomas and lymphomas is assumed.

Pemphigus vulgaris is a disease in which the body's defenses, the main task of the immune system, are directed towards the skin. As a result of this autoimmune disease, skin cells and skin tissue are damaged and ultimately destroyed.

Damage to the tissue can cause tissue fluid to appear and, in the case of pemphigus vulgaris, to collect in corresponding bubbles. Basically, two stages / phases are categorized in pemphigus vulgaris (initial phase and generalization phase).

causes

Of the Pemphigus vulgaris occurs relatively rarely, but is characterized by a severe course. In connection with the triggering causes of pemphigus vulgaris, no clear results are available up to the present. This makes treatment of the disease more difficult.

Not only tumors or carcinomas as well as the development of very special protein bodies can be the trigger for pemphigus vulgaris. Some selected active ingredients in drugs are also suspected of being able to promote pemphigus vulgaris.

In addition, possible causes of pemphigus vulgaris are damage to the skin due to the action of physical factors such as strong UV rays, X-rays and burns to the skin.

Symptoms, ailments & signs

General signs of pemphigus vulgaris are malaise, fatigue, and physical weakness. Some of those affected also suffer from fever, loss of appetite and weight loss. The typical symptom are skin vesicles of different sizes.

They prefer to form on mucous membranes. Most often this happens in the mouth. The soft pustules are filled with watery fluid. They are tender to pressure but not itchy. Your thin skin cracks very easily. This creates open, painful areas. The healing is slow. If the infection is more severe, the oral mucosa is comprehensively affected.

The disease can spread to the nose and throat. Additional burdens are the result. Eating, drinking and swallowing are clearly impaired. Other preferred areas of the body for bladder addiction are the hairy scalp, armpits, chest area and the genital and anal mucosa. The more the surface is affected, the more pain occurs.

When the top layer of skin heals, permanently damaged areas of skin remain underneath. As characteristic features, weeping or encrusted wound surfaces are formed at these points, which are constantly expanding. The complexion is comparable to a burn. Symptoms that occur less often are extensive skin peeling. They are often prone to widespread infections. In the worst case, these symptoms lead to life-threatening blood poisoning.

Diagnosis & course

The patients who are under Pemphiguss vulgaris suffer, are tormented by the fluid-filled skin blisters, which spread extensively.

Blisters are particularly common in pemphiguss vulgaris on the face, flanks, back and abdomen, as well as in the groins, buttocks and under the armpits. The first blisters in pemphigus vulgaris usually form in the mucous membrane of the mouth.

The development of large open skin defects in pemphigus vulgaris often causes purulent foci due to invading germs. Itchy crusts and scars also form. In extreme cases, those affected complain of fever, flu-like symptoms and a changed complexion. The discomfort of the skin subsides very slowly with pemphiguss vulgaris.

A special skin test is used to diagnose pemphigus vulgaris. In addition to the visual assessment of pemphigus vulgaris, microscopic examinations of the skin and an antibody titer determination can also be considered for diagnostic clarification.

Complications

Because of pemphigus vulgaris, patients primarily suffer from aesthetic complaints about the skin. This mainly leads to reddening of the skin and the formation of small blisters. These can be filled with a liquid and hurt. In general, pemphigus vulgaris significantly reduces and reduces the patient's quality of life.

Most of those affected also suffer from symptoms of the flu. There is a fever and loss of appetite. Weight loss and general weakness can also occur and have a negative effect on the everyday life of the patient. As a rule, the disease also leads to scars and thus to significant limitations in aesthetics. The patients no longer feel beautiful and may suffer from inferiority complexes or from a reduced self-esteem.

In children, the disease can lead to bullying or teasing. The symptoms of pemphigus vulgaris can be limited with the help of medication. There are no particular complications. In many cases, patients are dependent on prolonged use of these drugs. Life expectancy is usually not reduced with this disease.

When should you go to the doctor?

Skin changes that suggest pemphigus vulgaris or another form of pemphigus should be examined by your doctor. If yellowish crusts appear on the skin, erosions on the mucous membranes or accompanying symptoms such as fever, a doctor is recommended. Bleeding and infections must be clarified as quickly as possible, because if left untreated, the disease can spread to surrounding tissue. Blood-filled blisters, severe itching and abnormal sensations are warning signs that indicate a severe course and must therefore be examined and treated quickly.

Pemphigus vulgaris is treated by a family doctor or a dermatologist depending on the type and severity of the symptoms and their location. For mild forms, a pharmacist can be consulted, who will prescribe a suitable preparation after a brief consultation with the patient. If there are changes in the skin of the mouth, the dentist can be visited. People who develop pemphigus vulgaris in connection with a tumor disease or the use of aggressive medication should consult the responsible doctor.

Treatment & Therapy

The therapy of Pemphigus vulgaris is extremely tedious and is mainly based on medication. These are supposed to weaken the body's immune system. Specifically selected drugs against pemphigus vulgaris are so-called immunosuppressants and substances with a high cortisone content.

The therapy of pemphigus vulgaris always takes place in a complex of several procedures, which concerns both the causes and the occurring complaints. If the blistering caused by pemphigus vulgaris occurs in the mucous membrane of the mouth, it is advisable to use highly effective mouthwashes and avoid salty and spicy foods. These options against pemphigus vulgaris are supplemented by the treatment of inflamed skin regions caused by lesions with cortisone-containing ointment dressings.

The affected areas are kept moist with aseptic bandages in order to avoid the incrustations that occur in pemphigus vulgaris. Placing sterile pads with an iodine tincture can prevent bacteria from penetrating open wounds caused by pemphigus vulgaris. In some cases, pain relievers are also administered to enable food and drinks to be consumed.

Outlook & forecast

The course of the disease varies greatly from acute to chronic and depends on several factors. Since the introduction of cortisone therapy, there have been fewer fatal forms. If left untreated, pemphigus vulgaris can be fatal due to superinfections and cachexia (worldwide mortality of 5-10%), most frequently in the first 5 years after diagnosis. Long-term therapy with glucocorticoids and immunosuppressants and their side effects can also increase mortality. The skin disease occurs spontaneously and usually takes place chronically in phases over many months and years. However, there are also spontaneous remissions (= self-healing).

The course of the disease can be negatively influenced by the need for high cortisone doses and existing previous illnesses. A poor prognosis also results in elderly patients and extensive spread of the skin symptoms.

Early diagnosis and therapy start favor the progression of the blistering skin disease. After starting therapy, it takes an average of 2-3 weeks for the blisters to stop reappearing. The healing of the skin changes takes about 6-8 weeks. Close medical check-ups and dose adjustments of the required medication also result in a positive prognosis. Some studies show that 50% of patients are healthy or symptom-free after 5 years and 75% after 10 years.

prevention

Active prevention against Pemphigus vulgaris unfortunately does not exist. The development of pemphigus vulgaris is mainly observed in adults over the age of 30. A positive aspect of pemphigus vulgaris is that self-healing occurs in many cases, so that only a few people suffer from a chronic course of pemphigus vulgaris.

The frequency of dying from pemphigus vulgaris has decreased significantly with comprehensive medical treatment and care. This is also due to the fact that those affected usually seek medical treatment in good time.

Aftercare

With pemphigus vulgaris, those affected usually have only limited or only a few direct follow-up measures available. With this disease, those affected depend primarily on a quick diagnosis and subsequent treatment of the disease to relieve symptoms and prevent other complications. As a rule, there is no independent healing, so that continuous treatment by a doctor is necessary.

For the treatment itself, in most cases the person concerned is dependent on the intake of various medications. The correct dosage and regular intake should always be observed in order to relieve the symptoms permanently and above all properly. If anything is unclear or if you have any questions, you should always consult a doctor first.

Likewise, those affected by pemphigus vulgaris should protect their bodies particularly well and observe a high standard of hygiene in the case of wounds. Discomfort on the skin can be treated with creams or ointments, although regular checks by a dermatologist are also very useful. In many cases, however, the disease also significantly reduces the life expectancy of those affected.

You can do that yourself

This skin disease must be treated by a doctor, as a superinfection of the blisters can lead to life-threatening sepsis. The doctor may also find a cause such as an underlying disease to be treated. If medication is the cause of the disease, a change of medication may help.

Patients with pemphigus vulgaris should ensure adequate nutrition, even if they suffer from painful blisters in the mouth, nose and throat that make it difficult to eat. Since the body is weakened by the many small infections, the food should be light, rich in vitamins and easily digestible. Regular rest periods help against tiredness and feelings of weakness.

Since the disease can be very painful and can be accompanied by cosmetic impairments in the facial area, those affected often feel very stressed. You can benefit from accompanying psychotherapeutic treatment. It is also advisable to contact a self-help group. For example, a free membership in the “Pemphigus and Pemphigoid Self-Help e. V. “, because the association always provides the latest information on the disease online and patients can directly exchange information (www.pemphigus-pemphigoid-selbsthilfe.de).

If children are affected by the disease, it is possible that they are teased or even bullied in kindergarten and school. Parents should pay attention to this and take countermeasures if necessary.