Pemphigus foliaceus is an autoimmune disease of the skin in which cells of the immune system break down proteins connecting the skin. Cracks form on the skin, which later become blisters. Treatment is through the systemic administration of glucocorticoids or other drugs that suppress the immune system.
What is pemphigus foliaceus?
The human immune system recognizes pathogens and other substances of foreign origin as threats. After identification as foreign, the immune system launches attacks in order to eliminate any dangers to the organism as quickly as possible. In autoimmune diseases, this process is disrupted. See foodanddrinkjournal for Leigh Syndrome Dictionary Definitions.
The immune system of those affected attacks the body’s own tissue instead of foreign substances. Autoimmune diseases can attack any tissue. Doctors call those of the skin autoimmune dermatoses. Pemphigus foliaceus is an autoimmune dermatosis that causes blistering of the upper layer of the epidermis and can also affect mammals other than humans.
Medicine distinguishes four different forms of human pemphigus foliaceus: in addition to the sporadic pemphigus foliaceus type Cazenave, there is pemphigus braziliensis, which is mainly found in South America, pemphigus seborrhoicus and pemphigus erythematosus. As for most autoimmune diseases, the cause of the underlying immune dysfunction has not yet been finally clarified for pemphigus foliaceus.
Causes
Patients with pemphigus foliaceus produce autoantibodies directed against desmoglein 1. This is a protein within the desmosomes that is crucial for the cell cohesion of keratinocytes within the outermost layers of the skin. Through antigen-antibody reactions, the patient’s body releases proteolytic enzymes that break down proteins.
These enzymes break down the connections between individual skin cells with the proteins. As the skin lacks cell cohesion in a sequence, the keratinocytes round off. A so-called acantholysis occurs. Cracks then form in the epidermis, which later develop blisters. Desmoglein 1 occurs as a protein in only small amounts within the mucous membranes.
If it fails, its function in the mucous membranes is compensated for by desmoglein 3 in the upper layers of the mucous membrane. Because of this, the effects of pemphigus foliaceus are limited to the outer skin. It has not yet been clarified which factors are primarily involved in the incorrect programming of the immune system. For example, viral diseases that are the primary cause of autoimmune diseases are being discussed.
Symptoms, Ailments & Signs
Patients with pemphigus foliaceus have flaccid and quickly bursting blisters on the outer skin. The bottom of the blisters consists of weeping, crusted erosions that slowly expand and can cause generalized erythroderma. Erythroderma corresponds to redness that can affect the entire skin organ or larger parts of the skin.
In many cases, the erosions of patients with pemphigus foliaceus smell unpleasant. The secretion contained in the blisters is decomposed by bacteria, whose metabolic products are responsible for the bad smell. Some patients also complain of itching or burning spots on the skin. Because they rub the skin and scratch the blisters to cause itching, the blisters often spread uncontrollably.
The expanding bubbles are based on the principle of the positive Nikolski phenomenon. In most cases, the blisters affect the head, face, and trunk. Blisters form within the mucous membranes only in the rarest of cases.
Diagnosis & course of disease
The dermatologist makes the diagnosis of pemphigus foliaceus on the basis of serology and histopathology. The Nikolski signs can give rise to a first suspicion. The patient’s skin can be detached in the active stage by applying light tangential pressure, for example. Autoantibodies can be observed in the serum and in the intercellular space.
In later stages of the disease there is also accelerated blood sedimentation. In addition, the blood count changes. Dysproteinemia occurs. Histopathologically, the formation of acantholytic blisters primarily affects the stratum granulosum. Evidence of acanthosis, papillomatosis, or leukocyte infiltration can often be found within the dermis.
In terms of differential diagnosis, the disease must be differentiated from discoid lupus erythematosus and seborrheic eczema. In the detailed diagnosis, the diagnosis is narrowed down to one of the four forms of the disease. Like all other autoimmune diseases, pemphigus foliaceus is characterized by an individual course. This means that the prognosis for patients with the disease cannot be easily generalized.
Complications
Pemphigus foliaceus needs to be treated urgently, otherwise it can lead to multiple complications. Without treatment, the skin lesions caused by the body’s autoimmune reaction can spread throughout the body and cause what is known as generalized erythroderma. In generalized erythroderma, the entire skin becomes inflamed and reddened.
Characteristic of the disease process are heat and protein loss, increased cell proliferation of skin cells and the expansion of blood vessels. Serious complications, which can even become life-threatening, occur with particularly pronounced skin reactions. Severe dehydration can cause life-threatening dehydration. Dangerous protein and heat losses occur due to the increased formation of skin cells and extensive flaking of the skin.
Furthermore, the widening of the blood vessels often leads to serious cardiovascular diseases. The constant formation of bubbles and liquid also offers bacterial pathogens an ideal breeding ground. If left untreated, severe infections can develop, which can also be fatal. However, this danger can be 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 precaution during treatment to avoid serious infections. So far there is no curative treatment. The immune system must be constantly suppressed, so that precautions must be taken to avoid infectious diseases.
When should you go to the doctor?
Since pemphigus foliaceus does not heal itself and in most cases the symptoms worsen, treatment by a doctor makes sense. The symptoms can only be limited and alleviated by medical treatment. The doctor should be consulted for pemphigus foliaceus if blisters form on the skin, which do not disappear easily and appear over a longer period of time. Itching or severe redness on the affected area of skin can also indicate pemphigus foliaceus and should be examined by a doctor.
Since those affected by this disease often scratch themselves, the symptoms intensify. The blisters appear on different parts of the body and can make the patient’s life significantly more difficult. If you experience these symptoms, see a doctor immediately. As a rule, the disease pemphigus foliaceus can be diagnosed and treated by a general practitioner or a dermatologist. The life expectancy of the affected person is not negatively affected.
Treatment & Therapy
To date, the primary cause of pemphigus foliaceus has not been identified. For this reason, causal therapy is difficult. The disease is therefore considered an incurable disease with no causal therapy option.
However, symptomatic and supportive treatment methods are available for patient therapy. The symptomatic treatment of pemphigus foliaceus is essentially similar to the treatment of pemphigus vulgaris. Glucocorticoids are administered systemically.
Initially, high doses are adequate to arrest the disease process. Once Nikolsky’s 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.
Long-term immunosuppressive therapy is just as important as caring for the skin lesions. The administration of antibiotics is suitable for the prevention of complications. Rapid discontinuation of individual medications must be avoided as a matter of urgency. In this context, recurrences of symptoms were often documented.
Outlook & Forecast
If left untreated, the chronic autoimmune disease pemphigus foliaceus usually leads to death five years after the onset of the disease. Holistic corticosteroid treatment, immunosuppressants, plasma exchange, or immunoglobulin are often prescribed by the dermatologist to improve the prognosis. The treatments that are intended to lead to a reduction in the antibody titer are usually carried out on an inpatient basis and under supervision in order to counteract any complications, including fatal ones, that may arise during the therapy.
Since the 1950s, the use of corticosteroids over a period of one year has led to better long-term prospects for affected patients, so that up to 80% can continue to live free of symptoms or even fully recovered in the long term. Those affected are often restricted in their everyday activities during and after treatment, as they have to expect many hours of absence from work, weight loss and lack of sleep. The exchange with other affected people who have to go through the same thing can help them to cope with their change in life.
However, about 5% of cases still end fatally due to the consequences of treatment of systemic infections – more rarely after superinfection of the lesions – and lack of treatment. The sooner the disease is diagnosed and treatment started, the better the chances of those affected to live a symptom-free life.
Prevention
So far, no promising preventive measures against pemphigus foliaceus are available. The primary triggers of the autoimmune disease are not yet known. Only the identification and subsequent avoidance of the triggers could correspond to a preventive measure.
Aftercare
In the case of pemphigus foliaceus, the options for direct follow-up care are severely limited in most cases. The affected person is dependent on the quick and, above all, early diagnosis and treatment of this disease, so that no further complications occur and other symptoms do not arise.
Various medications are required. The doctor’s instructions must always be followed. It is also important to ensure that the dosage is correct and that it is taken regularly in order to relieve the symptoms properly and permanently.
When taking antibiotics, those affected should note that they should not be taken together with alcohol in order not to reduce their effect. Medication should not be discontinued quickly for pemphigus foliaceus. Regular check-ups and examinations by a doctor are also necessary to monitor the current state of the disease and to detect other damage at an early stage.
You can do that yourself
Pemphigus foliaceus can be relieved with cortisone, which the doctor prescribes as tablets or an infusion. When taking cortisone, patients should follow the recommended dosage exactly. The cortisone treatment protects against new inflammations, so that the skin erosions decrease. It is important not to irritate the affected skin unnecessarily. Side effects may occur when taking the preparations. This is exactly why the right dosage plays such an important role.
Patients can also use ointments or lotions to heal the damaged skin. You should definitely listen to the doctor’s recommendation. Careful, correct skin care prevents secondary inflammation. If there are other skin problems or autoimmune diseases, it is important to let the doctor know about them. In this way, he can help to make the ideal adjustment to the medication.
Of course, in order to protect the damaged skin, patients must not scratch themselves. Otherwise the problem will get worse. Calming exercises and a good body awareness help to let the spots heal in peace. In this way, those affected can make their lives a little easier. Allergy sufferers should be particularly careful and avoid the allergy trigger as much as possible.
