Diseases

Autoimmune Blistering Dermatoses – Causes, Symptoms & Treatment

Blistering autoimmune dermatoses

Blister-forming autoimmune dermatoses are autoimmune diseases in which the body’s own immune system recognizes the links between the skin layers as substances to be defended against. The defense system therefore destroys parts of the skin with antibodies and thus initiates the formation of blisters. The immune system of patients with autoimmune dermatoses is down-regulated as far as possible by means of long-term immunosuppressive treatment.

What are blistering autoimmune dermatoses?

The human immune system recognizes pathogens and other foreign substances that have penetrated the organism. Immunological cells then send antibodies to the affected area to ward off the hazardous substances. Various diseases can impair this body’s defense reaction.

Autoimmune diseases are immunological malfunctions. Cells of the immune system mistakenly 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 them is that of blistering autoimmune dermatoses. The immune system of people with blistering autoimmune dermatoses directs antibodies against components of the skin. The upper layers of the skin separate in a sequence from the underlying tissue. Blistering begins. Pemphigoid and pemphigus diseases are subtypes of this type of disease. The two types have different pathophysiology.

causes

The etiology of autoimmune diseases is generally considered to be unclear. In the past, various influences, including viruses and environmental toxins, have been discussed as the primary causal factors for immunological programming errors. The pathophysiology of blistering autoimmune dermatoses is relatively clear despite the unclear primary cause.

In pemphigoid diseases, antibodies are directed against protein molecules that connect individual layers of keratocytes to the basement membrane. This process detaches the basement membrane and promotes the formation of bulging blisters. Examples of such diseases are bullous pemphigoid, cicatricial mucosal pemphigoid and gestational pemphigoid.

In the case of pemphigus diseases, on the other hand, the antibodies attack desmosomes in the epidermis, which connect keratocytes to one another. Blistering is more subtle in these autoimmune dermatoses. In some cases, the upper layer of skin comes off like puff pastry. Examples of known pemphigus diseases are pemphigus vulgaris and pemphigus foliaceus.

Symptoms, Ailments and Signs

Patients with blistering autoimmune dermatoses suffer from different symptoms. In individual cases, the symptoms depend primarily on the subtype. Bulging blisters indicate pemphigoid disease. More subtle or absent bubbles speak more for pamphigus diseases.

Many autoimmune dermatoses are accompanied by itching or a slight burning sensation . This symptom motivates patients to scratch. However, the scratching process aggravates or spreads the dermatoses further. While in individual cases the mucous membranes can also be affected by blistering autoimmune dermatoses, this phenomenon is rather atypical for pamphigus diseases.

What all blistering dermatoses of an autoimmune nature have in common is the destruction of proteins, enzymes or other links within the layers of the skin. Which links the autoantibodies destroy in detail and in which layers these substances are located depends on the specific disease.

diagnosis and course

The dermatologist diagnoses a blistering autoimmune disease . The first suspicion arises purely from visual diagnostics. The suspicion is confirmed by immunofluorescence microscopy of skin particles or serum diagnostics.

The differentiation of individual autoimmune dermatoses with the naked eye can be difficult. In the histopathological analysis, however, the individual forms can be distinguished from one another relatively well. The detection of certain antibodies in the skin is a crucial step. For patients with autoimmune blistering dermatoses, the prognosis depends on the particular disease.

Personal factors also play a crucial role. Especially for autoimmune diseases, an individual course applies in principle. In addition, the treatment options for affected patients have improved significantly in recent years.

complications

Blister-forming autoimmune dermatoses describe certain dermatological diseases that attack the skin structure through antibodies from the body’s own household. Parts of the skin and tissue detach in layers and blisters form. The symptom occurs mainly on the extremities, upper body, face and mucous membranes.

For those affected, complications arise in everyday and professional life. Unpleasant pain symptoms and itching can become an additional physical and 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 vasodilatation of the skin structure along with blistering. Also rare is epidermolysis bullosa acquisita, where antibodies destroy collagen 7 and leave the skin with lesions and blisters.

Duhring’s disease reacts with flaking skin, persistent itching and blistering. If you suspect a blistering autoimmune dermatosis, you should consult your doctor immediately. Drug therapy is case-oriented and requires weakening of the immune system in order to contain the symptom.

Depending on the constitution of the patient, further complications can arise. In addition to corticosteroids, immunosuppressants are also given, which are not always tolerated. In an emergency, a blood wash for an antibody exchange must be initiated.

When should you go to the doctor?

Blister-forming autoimmune dermatoses always require a visit to the doctor. In this group of blistering skin diseases, the immune system attacks certain skin areas or skin layers. The affected skin forms blisters that eventually open.

Without a doctor’s visit, neither the cause of the blistering can be clarified, nor can professional treatment take place. This group of blistering skin diseases is classified as an autoimmune disease. This excludes successful self-treatment or the recognition of the trigger by the patient. Those affected are dependent on a competent doctor to put an end to their suffering from open blisters.

In many cases, a specialist doctor is only available to those affected after a long odyssey through various dermatological practices. The reason: bullous autoimmune diseases are among the rarer skin diseases. Such patients often only find help in the “Center for Rare Diseases” of the UKSH or similar clinical facilities.

Once the blister-forming autoimmune dermatoses have been correctly identified, a long-term improvement can usually be achieved through targeted treatment and immunosuppression. The people affected cannot be helped without histopathological or serological diagnostics. All attempts by those affected to cope without the help of a doctor fail.

Treatment & Therapy

Despite intensive research, the primary triggers for autoimmune diseases have not been finally clarified. Thus, essentially only speculations about the primary causes of the diseases can be made, a causal therapy remains unclear until the causal cure. Although modern treatments target the immune system, they do not reverse the programming errors.

Instead, the immune system is generally dampened by modern treatment approaches 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.

With immunosuppressants , the activity of the body’s immune system is down-regulated. Known immunosuppressants are, for example, various cortisone preparations that have a systemic effect. Drugs such as dapsone , azathioprine and mycophenolate mofetil are also used for long-term immunosuppressive treatment of blistering autoimmune dermatoses .

Acute therapy is often carried out with cortisone. 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 washing uses centrifugal forces and selectively separates the patient’s blood from the antibodies in order to return the filtered blood to the person concerned. Various drugs can also be used to inhibit the formation of new antibodies.

Since diet has been identified as a risk factor for some autoimmune dermatoses, diet should be adjusted if necessary. Avoiding stressful situations or using stress management strategies can also have a positive effect on your personal development.

Outlook & Forecast

The prognosis for blistering autoimmune dermatoses is unfavorable. Despite all efforts, scientists and researchers have not yet been able to find the cause of the disease. Targeted medical treatment is therefore not possible.

In practice, various therapeutic approaches are used to treat symptoms, but they do not lead to a cure of the disease. In most cases, long-term therapy takes place, since even months after the start of treatment, immediately after stopping the medication, the symptoms return immediately.

So far, no adequate results have been achieved with natural remedies or alternative healing methods. They can individually support the body and provide relief from various sequelae, but do not bring about a full recovery. Helpful are those against itching or to reduce stress . The patient experiences an improvement in general well-being.

As some research suggests that a solution can be found in a healthy immune system, there are efforts to stabilize and improve it. The patient can therefore experience relief from his symptoms with a healthy lifestyle and a balanced diet. Psychotherapeutic support for the patient helps to deal with the disease in everyday life . Although no cure is currently possible, a way can be found with the approaches 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 a disease. Eating habits as well as mental stress and viral diseases are considered risk factors for blistering autoimmune dermatoses. Although not all risk factors can be ruled out, there are hardly any other options for prevention, especially in the context of autoimmune diseases.

aftercare

In most cases, the measures or options for aftercare for this disease are very limited. First and foremost, the affected person is dependent on early detection of the disease so that further complications and a further deterioration of the symptoms can be avoided. The sooner a doctor is consulted, the better the further course of this disease.

For this reason, early detection is paramount. Patients with this disease are dependent on taking medication. It is important to ensure that it is taken correctly and regularly, and that the correct dose is also observed. If you have any questions or are unclear, you should always contact a doctor first.

It is not uncommon for those affected to be dependent on dialysis. They often also 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 those affected. In general, a healthy lifestyle with a healthy diet also has a positive effect on the further course of this disease.

You can do that yourself

From the point of view of conventional medicine, those affected can do little against the causes of the disease themselves, but they can avoid a number of mistakes that aggravate the course of the disease. Under no circumstances should patients scratch open the blisters. When scratching, there is a risk of scars forming, and germs can get into the scratch wound, which can lead to very unpleasant and sometimes dangerous secondary infections.

If the blisters itch badly, antihistamines from the pharmacy , which are available as creams, tablets or drops, can help. People who cannot control the scratching impulse should wear cotton gloves to avoid damaging the diseased skin with their fingernails. The blisters themselves cannot usually be made invisible with make-up, 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. Those affected by autoimmune diseases are therefore often advised to have their intestines cleansed and then cleaned up. The effectiveness of this treatment method has not been scientifically proven. However, patients repeatedly report healing successes. Since the treatment at least does not give reason to fear any serious side effects, there is nothing to be said against trying it.

The intestinal cleansing is usually accompanied by a change in diet, which is also advocated by conventional medicine. In any case, those affected should keep a food diary and check whether there is a statistical connection between certain foods and acute flare-ups of the disease.

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Hello! I am Lisa Newlon, and I am a medical writer and researcher with over 10 years of experience in the healthcare industry. I have a Master’s degree in Medicine, and my deep understanding of medical terminology, practices, and procedures has made me a trusted source of information in the medical world.