Pemphigus

Pemphigus is a rare disease of adults (40-60 years). Manifested by numerous bubbles (intraepidermally), erosions, scabs, and without treatment ends in death. In addition to changes in the skin and present in the oral mucosa in the form of erosion. In addition to local corticosteroid therapy, the patient is given high-dose systemic corticosteroids (prednisone / prednisolone, combined with the immunosuppressant azathioprine and mycophenolate mofetil.

ETIOLOGY:

The disease is of unknown etiology. As a causative agents are mentioned allergies and viral infections, as evidence that this dermatiza result of allergic reactions serves finding specific antibodies. Thus, in the blood serum and exudate are immunoglobulins belonging to the IgG class. Only in the early stage of the disease and IgA are detected. These antibodies are specific organs, because they react only with platelet-stratified epithelium.

CLINICAL:

The disease is characterized by formation of lumps on her skin unchanged or mucosa.Chronic pemphigus often affects the oral mucosa skin. It was found that in one third of patients, oral changes first occur in the oral mucosa, and in one third of the lumps appear in the mouth and on the skin. Very rarely, only 13% of patients, the changes do not affect the mouth and appear only on the skin and mucous membranes other. Primary efflorescence that occurs in the oral cavity is a lumps the size of grains of lentils, peas, hazelnuts, walnuts and a smaller to a chicken egg. lumps are filled with clear serous fluid pale more yellow caste. They occur in all parts of the oral mucosa. It is believed that the lumps rarely occur in the pink part of the lip and the gums. The roof lumps is very thin, so they quickly are sprayed, so that the former can not be seen in the mouth. After spraying lumps, there are surface erosion which sometimes can affect large parts of the oral epithelium. Severe damage are accompanied by increased salivation. Saliva is colored with blood, runny or drips from his mouth. The pain was very sharp, diet is difficult, because the liquid food irritates the damaged area.The disease progresses rapidly. Before using corticosteroids disease during two to three years always ended lethally. lumps the skin and mucous membranes nearby appear on the skin or mucous membranes unchanged. Practically there is no place where they do not occur.As a special predilective place for the emergence of lumps points out the umbilical and periubilikalni area.And lumps by the skin filled with clear serous fluid. Lumps on the skin lasting a few hours or days. Spraying lumps resulting erosion covered crust consisting of the dried secretions and the rest of the epidermis. Below the crust is formed epithelialization, and at that point you can re-occur lumps. Near the old bullous change occurs, further similar during the, resulting in large areas of skin are affected by the and half-naked. Changes to take months, the patient skinny and eventually dies. The diagnosis of oral pemphigus used Ni kolskijev (Nikolsky) phenomenon and Kolins (Tzank) ci-to diagnostic test.

PHENOMENON Nikolsky (Nikolsky) is corporeal test that helps determine the diagnosis of pemphigus.

During pemphigus (pemphigus vulgaris) weak and break the intercellular connections of the epithelium. This is highly emphasized in the spinous layer and such a phenomenon called acantholysis. The epidermis of the skin in patients with pemphigus are sepparating and the individual cells spinous layers are separated. Simultaneously, fluid accumulates in the epithelium, and thus form the lumps. The weakening of cells connection takes place throughout the skin and mucosa skins. Such a process does not have to manifest the appearance of lumps. During clinical examination, skin may appear unchanged. Nikolsky observed that, if the tissue, on skin seemingly unchanged, exert pressure, will soon be on the site form a lumps. This phenomenon has been used as an aid in determining the diagnosis of pemphigus.In a person with a suspected suffering from pemphigus chooses one region of the skin where there are lumps, and which looks unchanged. You could make sufficient pressure, this place should have a bone base. Such is for example podlekti - c ^. This place is pressed with your finger and then watched. Nikolsky phenomenon is positive if the epidermis at the site of pressure is separated, or if it forms a lumps. If you have already created a lumps exert pressure, lumps will increase. While it is useful in the diagnosis of pemphigus, the test Nikolsky not pathognomonic for the disease and can not be taken as a sure sign of pemphigus. This test can be positive for other skin diseases, especially those which lead to the weakening of the links in the epithelium and creating intraepithelial lumps.The lack of test Nikloskog is that is not positive throughout the disease, the most common is a positive at a time when the skin appear lumps.Niklosky phenomenon, despite the fact that is not a sure sign of pemphigus, if positive may facilitate the determination of diagnosis of pemphigus. A negative test does not rule Niklosky pemphigus and, if necessary, can be repeated many times. Chankov TEST is based on laboratory analysis of material removed from lumps.The epithelium of persons with pemphigus accumulating liquid, there arises the intercellular edema. Consequently weak and torn the intercellular connections. This particularly occurs between the spinous cell layer. This phenomenon is known as acantholytic lumps. In addition, the surface layers of the epithelium is separated from the spinous cells and the basal layer. Consequently lumps is localized near the basal .embrane, in the lower third of the epithelium. In lumps located called. damaged cells that the author called Canak cells. Such cells are circular in shape with a large sails. The essence Cank test lies in determining the presence of segregated cells in the material samples taken from lumps of the people patients with suspicion of being sick from pemphigus.Segregated Canak or cells are formed as a consequence of the described acantholysis, and degeneration, and epithelial changesin particular cells of the epithelial of layers, which are located near the basement membrane. Stratified squamous epithelia cells, which in healthy persons and stellate cells of the basal layer, which in healthy persons cube or a parallelepiped, in persons with pemphigus become globular. Such cells are called damaged cells, or cells Canak. They can be completely separate, but is more common in groups. Segregated cells have very the big mainsail, so it is very poor in the cytoplasm. The entire cytoplasm is pushed to the periphery and color are strongly basophilic. Hromatin is distributed diffusely. In the cytoplasm can clearly see the mainsail. The boundary between the sails and cytoplasm is clearly expressed. The sail of segregated cells not only a great, but the hype disrupted. often meet and exchange forms the sails. It may be doubled, twisted and changed in other ways. Determining the presence of segregated cell is performed in the material of the lumps. Select is fresh, unruptured lumps and its roof was punctured with a sharp instrument or needle. When the fluid has leaked, lumps is wide open. The material is taken from the bottom of ezom lumps. Material with loops is applied in a thin layer on glass slide for microscopy. made smear is dried in air. Painting smear is performed by the method of May-Grünwald (May-Gmvald) and Gimzo (Giemza). Plate of smear poured two minutes with May-Grinvaldovom color. To make the countdown, smear it during the three minutes overflows with distilled water whose electrochemical reaction neutral. Then he carried the overflow plate solution by Gimzo week three minutes. In order to remove excess paint, plate with smears are washed again for three minutes in distilled water neutral chemical reaction. preparation was air-dried and is then ready for testing Cytodiagnostical microscope. Microscopy is VTsIOM under immersion. when microscopic smear of lumps, it is necessary not only to ascertain the presence of damaged cells, but to determine their quality. The cells that are characteristic of pemphigus not only acantholytic and free, but also degenerate. In this manner, and the like differ from damaged cells in other diseases. Such cells for other diseases have not degenerated. Only degenerate acantholytic cells called segregovane cells.