Due to its appearance, the disease pemphigus vulgaris belongs in the category of dermatology. The optically visible manifestations of pemphigus vulgaris are limited exclusively to skin tissue.
What is pemphigus vulgaris?
The definition of pemphigus vulgaris refers to 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. See foodanddrinkjournal for Caudal Regression Syndrome Dictionary Definitions.
Pemphigus vulgaris is a disease in which the body’s defenses, the main task of the immune system, are directed at the skin. As a result of this autoimmune disease, damage and eventual destruction of skin cells and skin tissue occurs.
Tissue fluid can occur as a result of the damage to the tissue and collect in the corresponding blisters in pemphigus vulgaris. Basically, there are two stages/phases in pemphigus vulgaris (initial phase and generalization phase).
Causes
Pemphigus vulgaris is relatively rare, 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 also complicates the therapy of the disease in particular.
Pemphigus vulgaris can be triggered not only by tumors or carcinomas, but also by the development of very special proteins. Some selected active ingredients in medicines are also suspected of promoting pemphigus vulgaris.
In addition, damage to the skin caused by the effects of physical factors such as strong UV rays, X-rays and burns to the skin are equally possible causes of pemphigus vulgaris.
Symptoms, Ailments & Signs
General signs of pemphigus vulgaris are malaise, weakness and physical weakness. Some of those affected also suffer from fever, loss of appetite and weight loss. The typical symptom are skin blisters of different sizes.
They prefer to form on mucous membranes. This happens most often in the mouth. The soft pustules are filled with watery fluid. They are tender but not itchy. Their thin skin ruptures very easily. This creates open, painful spots. Healing is slow. If the infestation is more severe, the oral mucosa is extensively affected.
The disease can spread to the nose and throat. Additional burdens are the result. Eating, drinking and swallowing are clearly impaired. Other preferred parts of the body for bladder addiction are the hairy scalp, the armpits, the chest area and the genital and anal mucosa. The pain increases with increasing surface infestation.
When the top layer of skin heals, permanently damaged areas of skin remain underneath. Characteristic features are weeping or crusted wound surfaces that are constantly spreading. The complexion is comparable to a burn. Symptoms that occur less frequently are large areas of skin detachment. They are often prone to widespread infections. In the worst case, these symptoms lead to life-threatening blood poisoning.
Diagnosis & History
Patients suffering from pemphigus vulgaris are tormented by the fluid-filled skin blisters, which spread extensively.
The blisters in pemphigus vulgaris occur particularly frequently on the face, flanks, back and abdomen as well as in the groins, on the buttocks and under the armpits. In pemphigus vulgaris, the first blisters usually form in the mucous membrane of the mouth.
Due to the development of large open skin defects in pemphigus vulgaris, purulent foci often develop due to the invading germs. In addition, itchy crusts and scars form. In extreme cases, those affected complain of fever, flu-like symptoms and a changed complexion. The skin complaints in pemphigus vulgaris only subside very slowly.
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 skin problems. This mainly leads to reddening of the skin and the formation of small blisters. These can be filled with liquid and hurt. In general, pemphigus vulgaris significantly restricts and reduces the patient’s quality of life.
Most of those affected also suffer from symptoms of the flu. There is fever and loss of appetite. Weight loss and general weakness can also occur and have a negative impact on the patient’s everyday life. 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 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 taking these drugs for a long time. Life expectancy is usually not reduced with this disease.
When should you go to the doctor?
Skin changes that indicate pemphigus vulgaris or another form of pemphigus should be examined by your family doctor. If yellowish crusts on the skin, erosions on the mucous membranes or accompanying symptoms such as fever occur, a doctor’s visit is recommended. Bleeding and infections must be clarified as soon as possible, because if left untreated, the disease can spread to surrounding tissue. Blood-filled blisters, severe itching and abnormal sensations are also warning signs that indicate a serious course and must therefore be examined and treated quickly.
Depending on the type and severity of the symptoms and their localization, pemphigus vulgaris is treated by a general practitioner or a dermatologist. In the case of mild forms, a pharmacist can be consulted, who will prescribe a suitable preparation after a brief discussion with the patient. If skin changes occur in the mouth, the dentist can be consulted. People who develop pemphigus vulgaris in connection with a tumor or taking aggressive medication are best advised to consult the responsible doctor.
Treatment & Therapy
The therapy of pemphigus vulgaris is extremely lengthy and is mainly based on medication. These are supposed to weaken the body’s immune system. Targeted drugs against pemphigus vulgaris are so-called immunosuppressants and substances with a high cortisone content for this purpose.
The therapy of pemphigus vulgaris is always carried out in a complex of several procedures, which affects both the causes and the symptoms that occur. If blisters form in the mucous membrane of the mouth due to pemphigus vulgaris, it makes sense 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 bandages.
The affected areas are kept moist with germ-free bandages to avoid the crusting that occurs in pemphigus vulgaris. Placing sterile pads with a tincture of iodine on them can prevent bacteria from entering open wounds caused by pemphigus vulgaris. In some cases, painkillers are also administered to enable the intake of food and drinks.
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 deadly 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. The particularly long-term therapy with glucocorticoids and immunosuppressants and their side effects can also increase mortality. The skin disease occurs spontaneously, usually runs 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 required high doses of cortisone and existing pre-existing conditions. A poorer prognosis also results in old patients and extensive spread of the skin symptoms.
An early diagnosis and the start of therapy favor the course of the blistering skin disease. After the start of 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. In addition, close medical check-ups and dose adjustments of the required medication 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
Unfortunately, there is no active prevention against pemphigus vulgaris. The development of pemphigus vulgaris is observed primarily in adults over the age of 30. A positive aspect of pemphigus vulgaris is that self-healing often occurs, so that only a few people suffer from a chronic course of pemphigus vulgaris.
The frequency of dying from pemphigus vulgaris has fallen sharply with comprehensive medical treatment and care. This is also due to the fact that those affected usually seek medical treatment in good time.
Aftercare
In the case of pemphigus vulgaris, those affected usually have only limited or only a few direct follow-up measures available. Patients with this disease are primarily dependent on rapid diagnosis and subsequent treatment of the disease in order to relieve symptoms and prevent other complications. As a rule, self-healing cannot occur, so that continuous treatment by a doctor is necessary.
During the treatment itself, the affected person is dependent on taking various medications in most cases. The correct dosage and regular intake should always be observed in order to relieve the symptoms permanently and, above all, correctly. If anything is unclear or if you have any questions, you should always consult a doctor first.
Those affected by pemphigus vulgaris should also protect their bodies particularly well and observe a high standard of hygiene in the case of wounds. Skin complaints can be treated with the help of 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 because superinfection of the blisters can lead to life-threatening sepsis. The doctor may also find a cause of the disease, such as an underlying condition to be treated. If medication is the cause of the disease, changing the preparations may help.
Patients with pemphigus vulgaris should pay attention to an adequate diet, 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 also be accompanied by cosmetic impairments in the facial area, those affected often feel very stressed. Psychotherapeutic accompanying treatment can help you. It is also advisable to contact a self-help group. It is worthwhile, for example, to become a free member of the “Pemphigus und Pemphigoid Selbsthilfe e. V.”, because the association always puts the latest information about the disease online and patients can exchange information directly (www.pemphigus-pemphigoid-self-help.de).
If children are affected by the disease, they may be teased or even bullied in kindergarten and school. Parents should pay attention to this and take countermeasures if necessary.
