The disease pemphigus vulgaris belongs to the category of dermatology due to its appearance. The visually visible manifestations that occur in pemphigus vulgaris are limited exclusively to skin tissue.
What is pemphigus vulgaris?
Within the definition of pemphigus vulgaris, we speak of a so-called autoimmune disease of the skin, which is primarily associated with the expression of blisters. A causal connection of pemphigus vulgaris with autoimmune diseases of other organs as well as with carcinomas and lymphomas is assumed. Pemphigus vulgaris is a disease in which the body’s own defenses, the main function of the immune system, are directed at the skin. As a result of this autoimmune disease, there is damage and eventual destruction of skin cells and skin tissue. As a result of the damage to the tissue, tissue water may appear and collect in corresponding blisters in pemphigus vulgaris. Basically, two stages/phases are categorized in pemphigus vulgaris (initial phase and generalization phase).
Causes
Pemphigus vulgaris occurs relatively rarely but is characterized by a severe course. In the context of the triggering causes for pemphigus vulgaris, no clear results are available to date. This complicates in particular the therapy of the disease. Not only tumors or carcinomas as well as the development of very special protein bodies can be triggers of pemphigus vulgaris. Some selected active substances in medications are also suspected of promoting pemphigus vulgaris. In addition, damage to the skin by exposure to physical factors such as strong UV rays, X-rays, and burns to the skin are equally possible causes of pemphigus vulgaris.
Symptoms, complaints, and signs
Common signs of pemphigus vulgaris include malaise, faintness, and physical weakness. Some of those affected also suffer from fever, loss of appetite, and weight loss. The typical symptom is skin blisters of various sizes. They form preferentially on mucous membranes. This most frequently occurs in the mouth. The soft pustules are filled with watery fluid. They are sensitive to pressure, but do not itch. Their thin skin bursts very easily. This results in open, painful areas. Healing is slow. In more severe cases, the oral mucosa is extensively affected. The disease can extend to the nasal and pharyngeal cavities. Additional stress is the result. Eating, drinking and swallowing are significantly impaired. Other preferred body sites of cystitis are the hairy scalp, the armpits, the chest area, and the genital and anal mucosa. Increased pain occurs with increasing area infestation. When the top layer of skin heals, permanently damaged areas of skin remain underneath. Characteristic features of these areas are the formation of weeping or crusted sores that spread continuously. The skin appearance is comparable to a burn. More rarely occurring symptoms are extensive skin detachments. They are more often prone to extensive infections. In the worst case, these complaints lead to life-threatening blood poisoning.
Diagnosis and course
Patients suffering from pemphigus vulgaris are tormented by fluid-filled skin blisters that spread extensively. The blisters in pemphigus vulgaris are particularly frequent on the face, flanks, back and abdomen, as well as in the groin, buttocks and under the armpits. The first blisters in pemphigus vulgaris usually form in the mucous membrane of the mouth. The development of extensive open skin defects in pemphigus vulgaris often results in purulent foci caused by invading germs. Itchy incrustations and scars also form. In extreme cases, those affected complain of fever, flu-like symptoms and an altered skin appearance. The skin complaints subside only very slowly in pemphigus vulgaris. A special skin test is used in the diagnosis of pemphigus vulgaris. In addition, microscopic observations of the skin and antibody titer determination are considered for diagnostic clarification, in addition to visual assessment of pemphigus vulgaris.
Complications
Because of pemphigus vulgaris, patients primarily suffer from aesthetic complaints of the skin.The main symptoms are reddening of the skin and the formation of small blisters. These may be filled with a fluid and hurt. In general, the patient’s quality of life is considerably limited and reduced by pemphigus vulgaris. Most of those affected also suffer from symptoms of influenza. There is a fever and loss of appetite. Weight loss and general weakness may also occur and have a negative impact on the patient’s daily life. As a rule, the disease also leads to scars and thus to significant limitations in aesthetics. Patients may no longer feel beautiful and may suffer from inferiority complexes or lowered self-esteem. In children, the disease can thus lead to bullying or teasing. The symptoms of pemphigus vulgaris can be limited with the help of medication. This does not result in any particular complications. In many cases, patients are dependent on taking these medications for a long time. Life expectancy is not usually reduced with this disease.
When should you see a doctor?
Skin lesions suggestive of pemphigus vulgaris or another form of pemphigus should be examined by a primary care physician. If yellowish crusts appear on the skin, erosions on the mucous membranes, or accompanying symptoms such as fever, a visit to the physician 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 insensitivity are also warning signs that point to a severe course of the disease and must therefore be examined and treated quickly. Pemphigus vulgaris is treated by a general practitioner or a dermatologist, depending on the type and severity of the symptoms and their localization. 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 oral cavity, the dentist can be consulted. People who develop pemphigus vulgaris in connection with a tumor disease or the use of aggressive drugs are best to consult the responsible physician.
Treatment and therapy
The therapy of pemphigus vulgaris is exceedingly lengthy and is mainly based on medications. These should lead to weakening the body’s own immune system. Specifically selected drugs against pemphigus vulgaris for this purpose are so-called immunosuppressants and strongly cortisone-containing substances. The therapy of pemphigus vulgaris is always carried out in a complex of several procedures, which concerns both the causes and the occurring symptoms. If the blistering caused by pemphigus vulgaris occurs in the mucous membrane of the mouth, highly effective mouth rinses and avoidance of salty and spicy foods make sense. These options against pemphigus vulgaris are supplemented by the treatment of inflamed skin regions by lesions with ointment dressings containing cortisone. The affected areas are kept moist with germ-free dressings to avoid the crusting that occurs with pemphigus vulgaris. The application of sterile dressings with an iodine tincture can prevent bacteria from entering open wounds caused by pemphigus vulgaris. In some cases, analgesic medications are also administered to allow ingestion of food and drink.
Outlook and prognosis
The course of the disease varies widely from acute to chronic and depends on several factors. Since the introduction of cortisone therapy, there are fewer fatal forms. If left untreated, pemphigus vulgaris can be lethal due to superinfection and cachexia (worldwide mortality of 5-10%), most commonly in the first 5 years after diagnosis. Especially long-term therapy with glucocorticoids and immunosuppressants and their side effects can also increase mortality. The skin disease occurs spontaneously, usually progressing chronically in relapses over many months and years. However, there are also spontaneous remissions (=self-healing). The course of the disease can be negatively influenced by high cortisone doses and pre-existing diseases. A poorer prognosis also results in elderly patients and extensive spread of the skin symptoms. An early diagnosis and start of therapy favor the course of the blistering skin disease.After starting the therapy, it takes on average 2-3 weeks until the recurrence of the blisters stops. The healing of the skin changes takes about 6-8 weeks. Close medical check-ups and dose adjustments of the required medications also cause a positive prognosis. Some studies show that 50% of patients are healthy or free of appearances after 5 years and 75% after 10 years.
Prevention
Unfortunately, there is no active prevention of 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 in many cases it heals itself, so that only a few people suffer from a chronic course of pemphigus vulgaris. The incidence of dying from pemphigus vulgaris has greatly decreased due to comprehensive medical treatment and medical care. This is also due to the fact that sufferers usually seek medical treatment in a timely manner.
Aftercare
In pemphigus vulgaris, sufferers usually have limited or few measures of direct aftercare available. In this disease, affected individuals are primarily dependent on rapid diagnosis and subsequent treatment of the disease to alleviate symptoms and prevent other complications. As a rule, the disease cannot be cured on its own, so that continuous treatment by a physician is necessary. During the treatment itself, the affected person is in most cases dependent on taking various medications. The correct dosage and regular intake should always be observed in order to alleviate the symptoms permanently and, above all, correctly. If there are any uncertainties or questions, a doctor should always be consulted first. Likewise, those affected with pemphigus vulgaris should 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 the affected person.
What you can do yourself
It is imperative that this skin disease be treated by a physician, as superinfection of the vesicles can lead to life-threatening sepsis. The doctor may also find a cause of illness such as an underlying disease that needs to be treated. If medications are the cause of the disease, changing the medications may help. Patients with pemphigus vulgaris should pay attention to adequate nutrition, even if they suffer from painful vesicles in the mouth, nose and throat that make it difficult to eat. Since the body is weakened by the many small infections, food should be light, rich in vitamins and digestible. Regular rest helps against fatigue and feelings of weakness. Since the disease can be very painful and can be accompanied by cosmetic impairments, including in the facial area, those affected often feel very burdened. They can be helped by accompanying psychotherapeutic treatment. Contact with a self-help group is also recommended. For example, free membership in the “Pemphigus und Pemphigoid Selbsthilfe e. V.” (Pemphigus and Pemphigoid Self-Help Association) is worthwhile, as the association always provides the latest information on the disease online and patients can engage in a direct exchange (www.pemphigus-pemphigoid-selbsthilfe.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 initiate countermeasures if necessary.
