Description pemphigus

Pemphigus is difficult to treat, recurrent autoimmune bullous disease affecting the skin and mucous membranes throughout the body. It exists in several different forms, which differ in their manifestations, its localization, time of creation and by whom affect. It is caused by directing the immune system against its own structures, the structures of the skin, causing a change in the form of the formation of large and numerous blisters, its treatment is relatively complicated and not always completely successful.

Unlike previous times, and especially in the early interception, however, simplifies the whole procedure, and patients are able to live almost normally.


Risk factors

Pemphigus disease is not contagious, so you need not worry about being infected by any affected individual contact. There is no way to predict whether a person is in danger of developing serious illness, but in general we can say that more vulnerable people are middle-aged and older, and people of Jewish or Mediterranean descent. It is also believed that the increased risk in people who already suffer from the same time other autoimmune diseases, myasthenia gravis in particular, which is a chronic disease causing fatigue and weakness. Dangerous is also thymom- thymus tumor, thymus. It is an important part of the immune system and after the damage has, of course, can not perform its function well and gives space for the development of autoimmune diseases.


prevention pemphigus

For pemphigus neexstije no special prevention. It can only recommend, as with virtually any illness, adhere to the principles of a healthy lifestyle, eat well and play sports and avoid unnecessary stress.


Signs and symptoms of different types of pemphigus

pemphigus vulgaris

The most common and the most typical type of pemphigus is called pemphigus vulgaris. It is a chronic autoimmune disease arising from unknown causes. It manifests itself most often asinterapeidermální, thus penetrating inside the skin, blisters bull (bull’s bunion larger than five millimeters in diameter) conditional so-called Acantholysis: delaying the cells of the epidermis, or skin, and these spaces are then filled with fluid, which makes the formation of blisters. The delay cells occurs due to antibodies, immunoglobulins type G, directed against antigens on their keratinocytes membránách- desmoglein so-called third These antibodies can be demonstrated in the laboratory. For sowing these blisters is no typical place, they can occur anywhere on the body, both in the skin and mucous membranes, internal organs but they remain apart from similar diseases always unaffected. The appearance of blisters gradually changes from tougher with a net content to cloudy, the surrounding area is inflamed, after the bursting of erosion will continue changing to wet, slippery greyish scabs that smell strongly. Erosion itch, but burns and hurts very strongly. Disability is spreading rapidly, soon damaged and surrounding skin. There can not put more pressure to wipe erosion (likens it to peel the rind of an overripe peaches), this is called calleddirect Nikolsky’s sign. In addition, so-called. Nikolsky indirect flag means that when exerting pressure on the blister or bull comes to its magnification and enlargement and spilling into the surroundings. While on-site erosion scars arise, but in their place are formed early blistering new emerging suddenly, in atakách. If blisters occur on the oral mucosa, which is in most cases, is characterized by strong halitosis and increased salivation. It is also possible damage to the nail.Unfortunately the disease in its developed form, can end in death, especially in older people whose immunity has been reduced.

Most are diagnosed between the ages of forty and sixty years ago, they are very common among Jews.

pemphigus herpetiformis

Other, less common types include pemphigus herpetiformis pemphigus, which is often hard to distinguish from similar skin diseases such as mucous membrane pemphigoid and dermatitis herpetiformis. It creates a very itchy vesicle surface. Pemphigus vegetans against classical form milder, but unfortunately may change over time in vulgaris. Arises in damp locations Zápařka, blisters again strongly odorous and later merge exception is secondary infection yeast infection.Pemphigus foliaceus mainly affects middle-aged persons, typically extending it to the whole body and takes the form of peeling erythroderma (this is disabled completely the whole surface or skin surface of over 95 percent, at which the functions of the skin greatly reduced and the patient’s life-threatening). No formation when it blisters on the skin sloughing shreds, flakes, scabs, sowing strongly itches. Usually omits mucosa.

paraneoplastic pemphigus

Least common is the so-called paraneoplastic pemphigus, which is an accompanying phenomenon of various types of malignancy, usually lymphoma and Castleman’s disease (hyperproliferation, therefore, increased production of B lymphocytes in the lymph nodes). Sometimes it may even occur before the primary tumor is detected. Paraneoplastic pemphigus affect the oral mucosa, lips, esophagus, and even the lungs which causes known. Bronchiolitis obliterans, disease-causing inflammation closing bronchioles.

The damage is usually permanent and remain even after removal of the tumor and the healing of skin lesions.

In addition to typical skin and mucosal symptoms can occur in severe forms and the overall symptoms, such as temperature, schvácenost, chills, tremors, muscle and joint pain.


Examination and diagnosis

The diagnosis is typically determined by a dermatologist, who usually knows the disease according to the clearly visible signs or symptoms by Nikolských. In order to determine a diagnosis with certainty removes dermatopatolog biopsy of the affected skin, the area of the edge puchýře- this is called Tzankův test. In the microscope, the pathologist observes intraepidermal blister and clear signs Acantholysis. In conclusion, it is necessary to provide antibodies against desmoglein direct immunofluorescence. Antibodies are sorted podlél desmosomes, which are intercellular connections.Antibodies may also be afraid determined by ELISA.

It is important to distinguish from pemphigus pemphigus similar diseases, so-called pemphigoid.This is unlike pemphigus manifests as tougher blisters which are formed by separating the epidermis from škáry- blisters are therefore so-called subepidermal, unlike intraepidermálního pemphigus.


treatment of pemphigus

The most common method of treatment as well as many other autoimmune diseases is treatment with high doses of systemic corticosteroids such as prednisone. This causes depression of the immune system, including the part that attacks against its own cells and causes blisters. Typically, the course of treatment such that the first treatment is started very high doses of prednisone and 100 mg per day, and then gradually reducing the dose to a maintenance dose. Systemically administered steroids but have very serious and significant side effects, so we try therapy if possible replace. Therefore we use the auxiliary nekortikoidní immunosuppressants such asmycophenolic acid. It is also possible to use methylprednisolone, cyclosporine andazathioprine, classical and widely used immunosuppressants. After mastering worst stage of the disease, it is possible to gradually reduce the dose to prevent the development of serious side effects of these drugs. Often infection blisters in these cases are then prescribed tetracycline antibiotics. Basic drugs may be supplemented painkillers or powders which blisters zasypávají to prevent damage such as sleep.

Severe forms of pemphigus may require hospitalization, which is usually treated similarly to severe burns. Due to the loss of fluid from the blisters they are sometimes fluid delivered intravenously,as elektrolyty- minerals such as sodium, potassium or calcium- and protein. If the blisters in the mouth too painful, patients may be nourished intravenously and may have been administered anesthetics for relieving pain. It may also proceed to the therapeutic plasmoforéze in which blood plasma is separated from blood cells in order to rid the body of antibodies that attack it. Plasma is replaced by plasma from a donor.


How can I help myself

Pemphigus as a serious disease patient can not cope alone. But may help treat mainly disciplined compliance with prescribed medication dosing and carefully to avoid excessive damage to blistering which are then more easily become infected and may lead to sepsis. Totally inappropriate is therefore given, for example, contact sports.

Blisters can backfill all sorts of dusting or combat soothing emulsions.


complications of the disease

Pemphigus can cope treatment nevertheless occur during its course to various complications. One of these is the already mentioned infecting blisters that may be particularly serious if it occurs to a greater extent. This is called a generalized infection. Infection can also spread by blood and leads to the development of sepsis. Pemphigus danger also lies in the involvement of the oral mucosa, the patient can not eat for pain, and soon becomes malnourished and weakened, which in turn impedes the healing process. Often a patient suffering from dehydration. Paraneoplastic pembhigus complications are typically respiratory problems. The most serious complication is the death of the patient to that but nowadays there is only very, very heavy, neglected and untreated cases.

Other names: pemphigus, bullous disease

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