The immune system, which protects the person against disease-causing factors such as viruses and bacteria, easily recognizes any foreign substance that enters the body and develops a rapid defense mechanism against it. In this way, many harmful microorganisms that the person comes into contact with are removed from the body without causing a disease such as infection. The protection and defense functions of the immune system are extremely important in order to maintain a healthy life. However, sometimes some elements of this complex system perceive the body's healthy cells as foreign microorganisms, and in this case, the body develops an attack reaction against its own cells. This condition, which is called an autoimmune disease, can affect various tissues and organs of the body, especially the skin and joints. Pemphigus disease, which occurs with blisters filled with water in the skin and mucosal tissues, is one of the serious autoimmune diseases involving the skin.
What is Pemphigus?
The term pemphigus covers four different life-threatening bullous diseases characterized by loose bullae and cellular destruction in the skin and mucosal tissues. Basically, this disease group, which has four main clinical forms, is among the chronic autoimmune diseases that can lead to death. Pemphigus vulgaris is the most common form of the disease, which can occur in 4 different types: pemphigus vulgaris, pemphigus foliaceus, paraneoplastic pemphigus and IgA pemphigus. This form, which constitutes approximately 80% of all pemphigus cases, greatly affects the functioning of many body systems and can lead to death within 2 years if not treated.
What are the Symptoms of Pemphigus?
Pemphigus disease The complaints that may occur in the person with the disease can be summarized as follows:
- The disease usually occurs with wounds on the oral mucosa that do not heal for a long time. Mouth sores with a white surface, edematous around, and a slight pit in the middle are mostly seen at the tip of the tongue, but can also be seen on the inside of the lips, cheeks and palate. Long-lasting and frequently recurring oral aphthae are one of the most common symptoms of pemphigus disease, as in many autoimmune diseases. Severe mouth sores greatly affect the person's vital activities such as swallowing and feeding, resulting in inadequate health. It can lead to weight loss due to dehydration, weakening of the immune system and a decrease in body resistance. This situation not only disrupts the comfort of life of the person, but also makes the treatment of the disease difficult.
- Although the complaints related to pemphigus in the mouth give important clues about the disease, the most important symptom of pemphigus vulgaris is burn-like, water-filled blisters on the skin. These lesions, which mostly start on the scalp, progress rapidly and spread to the whole body, especially the face and trunk. Skin lesions, which initially appear as blisters that are filled with water and have the appearance of shriveled bubbles, are extremely sensitive and soon burst into open sores. These wounds, which become superficial and painful after bursting, dry and crust over time, but new wounds continue to appear. lesions may be overlooked. For example, the wounds that develop in the navel are not visible and only purulent discharge from this area is noticed. Apart from this, it is not possible to see the skin lesions under the nail tissue, but pain around the nail and a change in the shape of the nail tissue can be observed. Spread of oral lesions to the pharynx and esophagus causes pain during swallowing, hoarseness and intense nasal discharge especially in the morning, but all these are often overlooked symptoms of pemphigus vulgaris. and varies by organ. Involvement of the nose, eye and genital mucosa can be seen in addition to the oral mucosa. Tables in which the nasal mucosa are affected cause nasal congestion, bleeding and increased discharge, especially in the morning. In diseases with eye involvement, widespread redness of the eyes usually occurs. In case of involvement of the upper respiratory tract, complaints such as hoarseness and difficulty in breathing can be seen, while in cases of involvement of the esophagus, difficulty in swallowing and pain in the anterior chest wall may occur. In the involvement of the genital organs, bloody or purulent discharge is usually present. r.
It would be appropriate to suspect Pemphigus and to perform a biopsy, especially in cases of frequently recurring, unprovoked, aphthae-mixed mouth laws. For the real diagnosis of pemphigus, skin and mucosal biopsies for better immunohistochemical examination are required.
How is the Pemphigus Treatment?
Pemphigus disease, which is one of the rare autoimmune diseases, is not diagnosed in the early period and treated correctly. It is one of the diseases with a critical course that can lead to death if left untreated. The treatment of the disease is planned on a corticosteroid-centered basis, and additional diseases that cause the worsening of the course of the disease such as hypertension and diabetes are taken into account. Before the discovery of drugs called corticosteroids, pemphigus vulgaris was one of the diseases with an extremely high mortality rate, so this treatment option was a miracle for those with pemphigus. is evaluated. Drug treatment, which is started with the ideal application dose in the early period, eliminates almost all the complaints related to pemphigus and greatly increases the patient's life expectancy and quality of life. However, long-term use of corticosteroids in high doses, which is considered a medical miracle, can cause serious side effects in many systems of the body. In order to keep these side effects at the lowest level and to ensure that the patient receives the most accurate response to treatment, the “ideal corticosteroid dose” should be calculated according to the age, gender, the presence of chronic diseases such as hypertension and diabetes, the person's dietary habits and the severity of pemphigus vulgaris disease. Then, the patient is followed up with this treatment dose for a certain period of time and the side effects and the course of pemphigus-related complaints are evaluated. Since the ideal corticosteroid dose varies in line with these factors, regular control of the patient is extremely important for an effective treatment of pemphigus vulgaris. Even if your complaints have regressed, do not neglect to have regular check-ups for pemphigus disease.
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