Blistering autoimmune dermatoses are autoimmunologic diseases in which the body’s immune system recognizes connective tissue between the layers of the skin as substances to be rejected. The defense system therefore destroys parts of the skin with antibodies and thus initiates blistering. The defense system of patients is downregulated as much as possible with autoimmune dermatoses by means of long-term immunosuppressive treatment.
What are blistering autoimmune dermatoses?
The human immune system recognizes pathogens and other foreign substances that have invaded the body. Immunologic cells then send antibodies to the affected area to defend against the dangerous substances. Various diseases can impair this endogenous defense reaction. Autoimmune diseases are immunological malfunctions. Cells of the immune system falsely recognize the body’s own tissues as foreign and attack these tissues. In principle, all types of tissue can be affected by autoimmune diseases. For example, there are different subcategories of autoimmune diseases of the skin tissue. One of these is that of blistering autoimmune dermatoses. The defense system of people with blistering autoimmune dermatoses directs antibodies against skin components. The upper layers of the skin detach from the underlying tissue in a sequence. Blistering begins. Subtypes of this type of disease are pemphigoid and pemphigus. The two types have different pathophysiology.
Causes
The etiology of autoimmune diseases has generally been considered unexplained. Various influences have been discussed in the past as primary causative factors for immunologic misprogramming, including viruses and environmental toxins. The pathophysiology of blistering autoimmune dermatoses is relatively clear despite an unexplained primary cause. In pemphigoid disorders, antibodies target protein molecules that connect individual keratocyte layers to the basement membrane. This process detaches the basement membrane and promotes the formation of bulging blisters. Examples of such disorders include bullous pemphigoid, scarring mucous membrane pemphigoid, and pemphigoid gestationis. In pemphigus diseases, however, the antibodies attack desmosomes in the epidermis that interconnect keratocytes. Blistering is more subtle in these autoimmune dermatoses. In some cases, the upper layer of skin detaches in a flaky manner. Examples of known pemphigus disease include pemphigus vulgaris and pemphigus foliaceus.
Symptoms, complaints, and signs
Patients with blistering autoimmune dermatoses suffer from a variety of symptoms. In individual cases, symptoms depend primarily on the subtype. Bulging blisters indicate pemphigoid disease. More discreet or absent blisters are more indicative of pamphigus disorders. Many autoimmune dermatoses are associated with itching or mild burning. This symptom motivates patients to scratch. However, the scratching process further aggravates or spreads the dermatoses. While in individual cases the mucous membranes may also be affected by blistering autoimmune dermatoses, this phenomenon is rather atypical for pamphigus diseases in particular. Common to all blistering dermatoses of an autoimmunologic nature is the destruction of proteins, enzymes, or other connective members within the skin layers. The specific connective members that the autoantibodies destroy and the layers in which these substances are located depend on the specific disease.
Diagnosis and progression
The diagnosis of a blistering autoimmune disease is made by the dermatologist. The initial suspicion is purely by visual diagnosis. The suspicion is confirmed by immunofluorescence microscopy of skin particles or serum diagnostics. Differentiation from individual autoimmune dermatoses with the naked eye can be difficult. However, histopathologic analysis can distinguish the individual forms relatively well. The detection of certain antibodies in the skin is a decisive step in this process. For patients with blistering autoimmune dermatoses, the prognosis depends on the disease in question. Personal factors also play a decisive role. Particularly for autoimmune diseases, an individual course basically applies. In addition, treatment options for affected patients have improved significantly in recent years.
Complications
Blistering autoimmune dermatoses describe certain dermatologic conditions that affect the skin structure by antibodies from the body’s own immune system. In this process, parts of the skin and tissue detach layer by layer and blisters form. The symptom occurs mainly on the extremities, upper body, face and mucous membranes. For the affected person, complications arise in everyday and professional life. Unpleasant pain symptoms and itching can become an additional physical as well as psychological stress test. In dermatology, blistering autoimmune dermatosis is divided into groups. Pemphigoid diseases are directed against the connecting protein molecules with rather crusty skin detachment. The rare linear IgA dermatosis in children and adults shows a vasodilatation of the skin structure together with blistering. Also rare is epidermolysis bullosa acquisita, where antibodies destroy collagen 7 and affect the skin with lesions and blisters. Duhring’s disease reacts with skin scaling, persistent itching and blistering. If blistering autoimmune dermatosis is suspected, the physician should be consulted immediately. Drug therapy is case-oriented and requires weakening the immune system to contain the symptom. In this process, depending on the constitution of the patient, further complications may occur. For example, in addition to corticosteroids, immunosuppressants are also administered, which are not always tolerated. In an emergency, blood washing must be initiated for antibody replacement.
When should you see a doctor?
Blistering autoimmune dermatoses always require a visit to the doctor. In this group of blistering skin diseases, the immune system targets specific areas or layers of the skin. The affected skin forms blisters that eventually open. Without a visit to the doctor, neither the cause of the blistering can be clarified nor can professional treatment be given. This group of blistering skin diseases is classified as autoimmune diseases. This rules out successful self-treatment or recognition of the triggers by the patient. Affected individuals are dependent on an expert physician to put an end to their suffering from open blisters. In many cases, an expert physician is only available to sufferers after a long odyssey through various dermatology practices. The reason: bullous autoimmune diseases are among the rather rare skin diseases. Often such patients only find help in the “Center for Rare Diseases” of the UKSH or similar clinical facilities. Once the blistering autoimmune dermatoses are correctly classified, long-term improvement can usually be achieved by targeted treatment and immunosuppression. Affected individuals cannot be helped without histopathological or serological diagnosis. All attempts by affected individuals to cope without the help of a physician fail.
Treatment and therapy
Despite intensive research, the primary triggers for autoimmune diseases have not been conclusively clarified. Thus, in essence, only speculation can be made about the primary causes of the diseases, and causal therapy remains unresolved until a causal cure is found. Although modern treatments target the immune system, they do not reverse the faulty programming. Instead, modern treatment approaches basically dampen the immune system so that attacks on the body’s own tissue are more moderate in the future. Such treatment approaches are also known as immunosuppressive therapies. These are mostly conservative drug treatments. Immunosuppressants are used to downregulate the activity of the body’s immune system. Known immunosuppressants are, for example, various cortisone preparations that have a systemic effect. For long-term immunosuppressive treatment of blistering autoimmune dermatoses, agents such as dapsone, azathioprine, and mycophenolate mofetil are also used. Acute therapy is often with cortisone-elbows. Patients are instructed not to scratch the blisters. In addition, general skin care and hygiene is relevant in the context of autoimmune dermatoses. In particularly severe cases, the harmful antibodies can be removed from the blood using a special blood wash. This blood wash makes use of centrifugal forces and selectively separates the patient’s blood from the antibodies in order to return the filtered blood to the affected person.In addition, various drugs can be used to inhibit the formation of new antibodies. Since nutrition has been identified as a risk factor for some autoimmune dermatoses, the diet should be adjusted if necessary. Avoiding stressful situations or using strategies to cope with stress can also have a positive effect on the personal course.
Outlook and prognosis
The prognosis of blistering autoimmune dermatoses is unfavorable. Despite all efforts, scientists and researchers have not yet succeeded in finding the cause of the disease. Therefore, a targeted medical treatment is not possible. In practice, various therapeutic approaches are used to treat the symptoms, but these do not lead to a cure of the disease. In most cases, a long-term therapy takes place, because even months after the start of treatment, immediately after the discontinuation of medication, the symptoms return. Natural remedies or alternative healing methods have also not been able to achieve sufficient results to date. They can individually support the body and provide relief from various sequelae, but cannot provide complete recovery. Helpful are those against itching or for stress reduction. Thus, the patient experiences an improvement in general well-being. Since some research results suggest that a solution can be found in a healthy immune system, there are efforts to stabilize and improve it. Therefore, the patient may experience relief from his or her symptoms with a healthy lifestyle and a balanced diet. To deal with the disease in everyday life, psychotherapeutic support of the patient helps. Although no cure is currently possible, the approaches can be used to find a way to achieve a good quality of life despite the blistering autoimmune dermatoses.
Prevention
Preventive measures eliminate risk factors that could contribute to the development of disease. In blistering autoimmune dermatoses, dietary habits as well as psychological stress and viral diseases are thought to be risk factors. Although not all risk factors can necessarily be excluded, there are few other options available for prevention specifically in the context of autoimmune diseases.
Follow-up
In most cases, the measures or options for aftercare in this disease are very limited. First and foremost, the affected person is thereby dependent on an early detection of the disease, so that further complications and a further worsening of the symptoms can be avoided. The earlier a doctor is consulted, the better the further course of the disease usually is. For this reason, early detection is a priority. Patients with this disease are dependent on taking medication. It is important to ensure that the medication is taken correctly and regularly, and the correct dose must also be observed. In case of questions or uncertainties, a doctor should always be contacted first. However, it is not uncommon for those affected to be dependent on dialysis. They often need the help and support of friends and family, and psychological support is also very important. In most cases, this disease also reduces the life expectancy of the affected person. In general, a healthy lifestyle with a healthy diet also has a positive effect on the further course of this disease.
This is what you can do yourself
Affected individuals can do little about the causes of the disease themselves from a conventional medical perspective, but they can avoid a number of mistakes that exacerbate the course of the disease. Under no circumstances should patients scratch open the blisters. Scratching them open firstly runs the risk of scarring, and secondly germs can get into the scratch wound, which can lead to very unpleasant and sometimes dangerous secondary infections. If the blisters are very itchy, antihistamines from the pharmacy, which are offered as creams, tablets or drops, can help. People who cannot control the scratching impulse should wear cotton gloves so that they do not injure the diseased skin with their fingernails. The blisters themselves usually cannot be made invisible with makeup, but at least the redness can be concealed so that the skin changes become less noticeable.In naturopathy, a connection between the autoimmune system and intestinal health is suspected. People affected by autoimmune diseases are therefore often advised to undergo a colon cleanse followed by intestinal rehabilitation. The effectiveness of this treatment method is not scientifically proven. However, patients repeatedly report healing successes. Since the treatment at least does not cause any serious side effects, there is nothing to be said against trying it. Intestinal rehabilitation is usually accompanied by a change in diet, which is also advocated by conventional medicine. Those affected should in any case keep a food diary and check whether there is a statistical correlation between certain foods and acute episodes of the disease.
