Pemphigus foliaceus is an autoimmunologic disease of the skin in which cells of the immune system break down proteins that bind to the skin. This causes clefts to form on the skin, which later become vesicles. Treatment is by systemic administration of glucocorticoids or other drugs that dampen the immune system.
What is pemphigus foliaceus?
The human immune system recognizes pathogens and other substances that are foreign to the body as threats. After identification as foreign to the body, the immune system drives attacks to eliminate any threats to the organism as quickly as possible. In autoimmune diseases, this process is disturbed. The immune system of affected individuals attacks the body’s own tissue instead of foreign substances. Autoimmune diseases can be directed against any tissue. Such diseases of the skin are called autoimmune dermatoses. Pemphigus foliaceus is an autoimmune dermatosis that causes blistering of the upper epidermal layer and can affect other mammals as well as humans. For human pemphigus foliaceus, medicine distinguishes four different forms: In addition to sporadic pemphigus foliaceus type Cazenave, there is pemphigus braziliensis, which is predominantly found in South America, pemphigus seborrhoicus and pemphigus erythematosus. As is the case for most autoimmune diseases, the cause of the underlying immune dysfunction has not yet been conclusively determined for pemphigus foliaceus.
Causes
Patients with pemphigus foliaceus form autoantibodies directed against desmoglein 1. This is a protein within desmosomes that is critical for cell cohesion of keratinocytes within the outermost layers of the skin. Antigen-antibody reactions cause the patient’s body to release proteolytic enzymes that degrade proteins. These enzymes break down the connections between individual skin cells along with the proteins. As the skin lacks cell cohesion in a consequence, the keratinocytes round off. A so-called acantholysis occurs. As a result, clefts form in the epidermis, which later blister. Desmoglein 1 occurs as a protein only in small proportions within the mucous membranes. Its function in the mucous membranes is compensated by desmoglein 3 of the upper mucosal layers in case of failure. For this reason, the effects of pemphigus foliaceus are limited to the outer skin. Which factors are primarily involved in the misprogramming of the immune system is still unclear. Primary causative viral diseases, for example, are discussed for autoimmune diseases.
Symptoms, complaints, and signs
Patients with pemphigus foliaceus have leading symptomatically flaccid and rapidly bursting blisters on the outer skin. The base of the blisters consists of weeping, crusty erosions that may slowly expand and cause generalized erythroderma. Erythroderma corresponds to redness that may affect the entire skin organ or larger parts of the skin. In many cases, the erosions of patients with pemphigus foliaceus have an unpleasant odor. The secretion contained in the blisters is decomposed by bacteria, whose metabolic products are responsible for the bad odor. Some patients additionally complain of itching or burning skin. As they rub over the skin and scratch at the blisters because of the itching, the blisters often spread uncontrollably. In this case, the spreading blisters are due to the principle of positive Nikolski phenomenon. In most cases, the blisters affect the head, face and trunk of the body. Within the mucous membranes, blister formation occurs only in the rarest cases.
Diagnosis and course of the disease
The dermatologist makes the diagnosis of pemphigus foliaceus based on serology and histopathology. An initial suspicion may be based on the Nikolski signs. In the active stage, the patient’s skin can be detached by light tangential pressure, for example. Autoantibodies can be observed in the serum and intercellular space. In later stages of the disease, accelerated blood sedimentation is also present. In addition, the blood count changes. Dysproteinemias occur. Histopathologically, acantholytic blistering primarily affects the stratum granulosum. Within the dermis, evidence of acanthosis, papillomatosis, or leukocyte infiltration can often be seen.Differentially, the disease must be differentiated from discoid lupus erythematosus and seborrheic eczema. In the fine diagnosis, the diagnosis is narrowed down to one of the four forms of the disease. Pemphigus foliaceus, like all other autoimmune diseases, is characterized by an individual course. Thus, the prognosis for patients with the disease cannot be readily generalized.
Complications
Pemphigus foliaceus must be treated urgently because otherwise it can lead to several complications. For example, the skin lesions caused due to the body’s autoimmune response can spread throughout the body without treatment and cause what is known as generalized erythroderma. In generalized erythroderma, the entire skin becomes inflamed and red. Characteristic of the disease process are heat and protein loss, increased cell proliferation of skin cells and dilation of blood vessels. Serious complications, which can even become life-threatening, occur with particularly pronounced skin reactions. For example, severe fluid loss can cause life-threatening dehydration. Dangerous protein and heat losses occur due to the increased formation of skin cells and extensive scaling of the skin. Furthermore, the dilation of blood vessels often leads to severe cardiovascular disease. The constant formation of blisters and fluid also provides an ideal breeding ground for bacterial pathogens. Thus, if left untreated, serious infections can develop, which may even be fatal. However, this danger can be further increased with the administration of immunosuppressants. However, since pemphigus foliaceus can only be treated by suppressing the immune system, antibiotics are usually given as a precautionary measure to prevent severe infections during treatment. So far, there is no curative treatment available. The immune system must be constantly suppressed, so precautions must also be taken constantly to prevent infectious diseases.
When should you go to the doctor?
Since pemphigus foliaceus does not heal itself and, in most cases, intensifies the symptoms, treatment by a doctor is advisable. Only medical treatment can limit and alleviate the symptoms. A doctor should be consulted in cases of pemphigus foliaceus when blisters form on the skin, which do not disappear easily and occur over a long period of time. Likewise, itching or a strong reddening on the affected skin area can indicate pemphigus foliaceus and should be examined by a doctor. Since those affected by this disease often scratch themselves, the symptoms are intensified. The blisters appear on different parts of the body and can make the patient’s life much more difficult. In case of these complaints, a doctor should be consulted immediately. As a rule, the disease pemphigus foliaceus can be diagnosed and treated by a general practitioner or by a dermatologist. The life expectancy of the affected person is not negatively affected.
Treatment and therapy
To date, the primary trigger for pemphigus foliaceus has not been identified. For this reason, causal therapy is difficult. Therefore, the disease is considered incurable with no causal therapy option. However, symptomatic and supportive treatment methods are available for the therapy of patients. Essentially, symptomatic therapy of pemphigus foliaceus is similar to the treatment of pemphigus vulgaris. Systemically, glucocorticoids are administered. Initially, high doses are appropriate to arrest the disease process. Once the Nikolski signs are negative and the skin defects begin to heal, a gradual reduction in dose is indicated. In addition to glucocorticoids, patients may receive other immunosuppressants. Continuous immunosuppressive therapy is as important as care of the skin lesions. For the prevention of complications, the administration of antibiotics is suitable. Rapid discontinuation of individual drugs should be avoided urgently. In this context, recurrences of symptoms have been frequently documented.
Outlook and prognosis
Without treatment, the chronic autoimmune disease pemphigus foliaceus usually leads to death five years after onset. To improve prognosis, dermatologists often prescribe holistic corticosteroid treatment, immunosuppressants, plasma exchange, or immunoglobulin.The treatments, which are intended to reduce antibody titers, are usually carried out on an inpatient basis and under supervision, in order to counteract any complications – including lethal ones – that may arise during the therapy. Since the 1950s, the use of corticosteroids over a period of one year in particular has led to better long-term prospects for affected patients, so that up to 80% can continue to live symptom-free or even fully recovered in the long term. Often, affected patients are restricted in their daily activities during and after treatment, with many hours of absence from work, weight loss and lack of sleep. In this regard, sharing their experiences with other affected individuals who are going through the same thing can help them cope with their life changes. However, about 5% of cases still end fatally due to the consequences of treatment systemic infections – rarely after superinfection of the lesions -, as well as lack of treatment. The sooner the disease is diagnosed and treatment started, the better the prospects for those affected to live symptom-free.
Prevention
To date, no promising preventive measures against pemphigus foliaceus are available. The primary triggers of the autoimmune disease are not yet known. Only identification and subsequent avoidance of the triggers could correspond to a preventive measure.
Follow-up
In most cases of pemphigus foliaceus, the options for direct follow-up are severely limited. The affected person is dependent on the rapid and, above all, early diagnosis and treatment of this disease, so that no further complications occur and other complaints do not arise. It is necessary to take various medications. The doctor’s instructions must always be followed. Likewise, it is necessary to pay attention to a correct dosage and also to a regular intake in order to relieve the discomfort properly and permanently. When taking antibiotics, sufferers should note that they should not be taken together with alcohol in order not to reduce their effect. Medications should not be discontinued quickly in pemphigus foliaceus. Regular checkups and examinations by a physician are also necessary to monitor the current state of the disease and to detect other damage at an early stage.
Here’s what you can do yourself
Pemphigus foliaceus can be relieved with cortisone, which the doctor prescribes as tablets or infusions. When taking cortisone, patients should follow the dosage recommendation exactly. The cortisone treatment protects against new inflammations so that the skin erosions recede. It is important not to irritate the affected skin areas unnecessarily. Side effects may occur when taking the preparations. This is precisely why the correct dosage plays such an important role. Patients can also use ointments or lotions to heal the damaged skin. In doing so, they should definitely listen to the doctor’s recommendation. Careful, correct skin care prevents secondary inflammation. If other skin problems or autoimmune diseases are present, the doctor should definitely know about them. In this way, he can help to make the ideal adjustment of the medication. In order to protect the damaged skin, patients must not scratch themselves, of course. Otherwise, the problem will worsen. Calming exercises and good body awareness help to let the spots heal in peace. In this way, sufferers can make life a little easier for themselves. Allergy sufferers should be especially careful and avoid the allergen as much as possible.
