Stephens-Johnson's syndrome
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Description:
Stephens's syndrome - Johnson (a malignant exudative erythema) very severe form of a mnogoformny erythema at which there are bubbles on a mucous membrane of an oral cavity, a throat, eyes, generative organs, other sites of skin and mucous membranes.
Injury of a mucous membrane of a mouth prevents to eat, closing of a mouth causes severe pain that leads to hypersalivation. Eyes become very painful, swell up and are filled with pus so that eyelids sometimes stick together. Corneas are exposed to fibrosis. The urination becomes complicated and painful.
Symptoms of the Syndrome of Stephens-Johnson:
This pathology always develops at the patient very quickly, promptly as in fact is allergic reaction of immediate type. In the beginning appear the most severe fever, a joint pain and muscles. Further, only several hours or days later, damage of a mucous membrane of an oral cavity comes to light. There are bubbles enough big sizes, defects of skin covered with films of gray-white color, the crusts consisting of clots of clotted blood, a crack. There are also defects in the field of a red border of lips. Damage of eyes proceeds as conjunctivitis (an inflammation of mucous eyes), however inflammatory process has purely allergic character here. Further can join and bacterial damage owing to what the disease begins to proceed more hard, a condition of the patient sharply worsens. On a conjunctiva at Stephens-Johnson's syndrome there can also be small defects and ulcers, the inflammation of a cornea, back departments of an eye can join (retinas of vessels, etc.). The defeat centers very often can take also generative organs that is shown in the form of an urethritis (an urethra inflammation), a balanitis, a vulvovaginitis (an inflammation of female external genitals). Mucous membranes in other places are sometimes involved. As a result of damage of skin on it a large number of spots of reddening with the eminences located on them over skin level as blisters is formed. They have roundish outlines, crimson coloring. In the center they cyanotic and as if sink down a little. Diameter of the centers can make from 1 to 3-5 cm. In the central part of many of them bubbles which contain transparent watery liquid or blood inside are formed.
After opening of bubbles on their place there are defects of skin of bright red color which then become covered by crusts. Generally the centers of defeat are located on a trunk of the patient and in a crotch. Disturbance of the general condition of the patient which is shown in the form of severe fever, an indisposition, weakness, fatigue, a headache, dizziness is very expressed. All these manifestations continue on average about 2-3 weeks. In the form of complications during a disease pneumonia, a diarrhea, insufficiency of function of kidneys, etc. can join. At 10% of all patients these diseases proceed very hard and lead to death.
Reasons of the Syndrome of Stephens-Johnson:
Development of allergic reaction in response to reception of antibiotics and other antibacterial drugs is the main reason for emergence of a syndrome of Stephens-Johnson. Now the hereditary mechanism of development of pathology is considered very probable. Genetic disorders in an organism are resulted by suppression of its natural protection. Not only skin, but also the blood vessels feeding it is surprised at the same time. These facts also cause all developing clinical displays of a disease.
Treatment of the Syndrome of Stephens-Johnson:
Drugs of adrenal hormones in average dosages are generally used. They are entered to the patient until comes permanent considerable improvement of a state. Then the dosage of drug begins to be lowered gradually, and in 3-4 weeks it is completely cancelled. At some patients the state is so heavy that they are not capable to accept drugs independently through a mouth. In these cases hormones are entered in liquid forms intravenously. Procedures which are directed to removal from an organism of the cell-bound immune complexes circulating in blood representing the antibodies connected with antigens are very important. For this purpose use special drugs for intravenous administration, methods of purification of blood in the form of hemosorption and a plasma exchange. Also the drugs accepted through a mouth, promoting removal of toxic substances from an organism through intestines are used. For the purpose of fight against intoxication daily not less than 2-3 l of liquid have to be entered into an organism of the patient in various ways. At the same time watch that all this volume was timely brought out of an organism as at a delay of liquid toxins are not washed away and rather heavy complications can develop. It is clear, that full implementation of these actions is possible only in the conditions of chamber of an intensive care. Quite efficient action is intravenous transfusion to the patient of solutions of proteins and human plasma. Drugs, calciferous, potassium, antiallergic medicines are in addition appointed. If the defeat centers very big, the condition of the patient rather heavy, then always exists risk of development of infectious complications which can be prevented by purpose of antibacterial agents in combination with antifungal drugs. For the purpose of treatment of skin rashes locally apply on them various cream, the containing drugs of adrenal hormones. Various solutions of antiseptic agents are applied to prevention of infection.
