Stevens-Johnson syndrome
Stevens-Johnson syndrome is an acute bullous lesion of the mucous membranes and skin, related to an allergic nature. Usually, the disease occurs against the background of severe conditions involving the oral mucosa, genitourinary organs and eyes. It is possible to diagnose Stevens-Johnson syndrome by a thorough examination of the patient, blood tests by the immunological method, thanks to skin biopsy and coagulogram. Taking into account the varying severity of the indications of the disease, lung X-rays, kidney ultrasound, bladder ultrasound, biochemical urine analysis, and of course, consultation with other specialists are carried out. Treatment is carried out by extracorporeal hemocorrection, through infusion therapy and glucocorticoid therapy. Antibacterial drugs are also prescribed.
The content of the article:
Causes of Stevens-Johnson syndrome
Symptoms of Stevens-Johnson syndrome
Diagnosis of Stevens-Johnson syndrome
Treatment of Stevens-Johnson syndrome
Stevens-Johnson syndrome
The first data on Stevens-Johnson syndrome were published only in 1922. Over time, Stevens-Johnson syndrome got its name in honor of its discoverers, whose names the disease was subsequently named after. This ailment is a variant of multiform exudative erythema, occurring in a severe variant. The disease also has a second name — malignant exudative erythema. Stevens-Johnson syndrome refers to bullous dermatitis, just like Lyell’s syndrome, allergic contact dermatitis, pemphigus, Haley-Haley disease, bullous variant of SLE and more. Interestingly, a common symptom of all of the above diseases, including Stevens-Johnson syndrome, is the appearance of blisters on the surface of the skin and on the mucous membranes.
Stevens-Johnson syndrome is detected at very different ages. However, it is most common in people from 20 to 40 years old, but very rare in the first three years of a child’s life. The prevalence of Stevens-Johnson syndrome ranges from 0.5 to 5-6 cases per 1 million people per year. Many researchers note that the disease is more inherent in men.
Causes of Stevens-Johnson syndrome
Stevens-Johnson syndrome develops under the influence of allergic reactions of a rapid type. There are only four groups of factors that can provoke the occurrence of the disease:
medicines;
infections;
malignant diseases;
causes of unknown nature.
In childhood, Stevens-Johnson syndrome is very often formed against the background of viral diseases, such as viral hepatitis, measles, influenza, herpes simplex, chickenpox, mumps. The main provocateur may be a bacterial factor (tuberculosis, mycoplasmosis, salmonellosis, tularemia, yersiniosis, brucellosis), as well as a fungal cause (trichophytia, coccidiomycosis, histoplasmosis).
In adulthood, Stevens-Johnson syndrome is explained by the introduction of medications, as well as a possible ongoing malignant process. Of medicines, the main cause may be antibiotics, anti-inflammatory nonsteroidal drugs, sulfonamides and a central nervous system regulator.
Interestingly, carcinomas and lymphomas play a leading role in oncological diseases and in the development of Stevens-Johnson syndrome. If it is impossible to establish an etiological factor, we are talking about the ideopathic Stevens-Johnson syndrome.
Symptoms of Stevens-Johnson syndrome
The onset of Stevens-Johnson syndrome is characterized by rapidly unfolding symptoms. At the very beginning , it is usually noted:
general malaise with a rise in body temperature to 40 ° C;
characteristic headache;
arthralgia;
tachycardia;
muscle aches;
may have a sore throat;
a cough may appear;
vomiting;
diarrhea.
After a couple of hours (maximum — a day later), large bubbles appear on the oral mucosa. After opening such bubbles, extensive defects appear on the mucous surfaces, which are covered with a film of white-gray or yellow color. There are also crusts formed from dried blood. The lip border may be involved in this pathological process. Due to severe damage to the mucous membranes in Stevens-Johnson syndrome, patients are not even able to drink or eat.
Effect on the eyes
The lesion of the eyes is very similar in its characteristics to allergic conjunctivitis, however, there is often a complication by secondary infection with the further development of inflammation with purulent content. Stevens-Johnson syndrome is also characterized by erosive and ulcerative elements formed on the cornea and conjunctivitis. Such elements are usually of insignificant size. It is also possible to damage the irises, the development of iridocyclitis, blepharitis and keratitis.
Defeat of the mucous membranes
Infection of the mucous membranes of the genitourinary system occurs in 50% of cases of this syndrome. The lesion is expressed by urethritis, vulvitis, balanoposthitis, vaginitis. Usually, scarring of erosions and ulcers of the mucous membranes entails the formation of urethral stricture.
How the skin is affected
The affected skin is represented by a significant number of rounded elements with elevation. Such elevations resemble blisters and have a purple color, reaching 5 cm. The peculiarity of the elements of such a skin rash in the case of Stevens-Johnson syndrome is the occurrence of blood and serous blisters in the center. If such bubbles are opened, bright red defects covered with crusts are formed. The main place of rashes is the skin of the perineum and trunk.
The time of occurrence of new rashes in Stevens-Johnson syndrome lasts approximately 3 weeks, and healing lasts 1.5-2 months. It should be said that the disease is complicated by pneumonia, blood discharge from the bladder, colitis, bronchiolitis, secondary bacterial infection, acute renal failure and even loss of vision. Due to developing diseases, about 10-12% of patients with Stevens-Johnson syndrome die.
Diagnosis of Stevens-Johnson syndrome
The doctor can identify Stevens-Johnson syndrome based on the presence of specific symptoms that can be detected by an experienced dermatological examination. By interviewing the patient himself, you can determine the cause that caused the development of this ailment. A skin biopsy will allow you to confirm the diagnosis itself. During histological examination, necrosis of epidermal cells may occur, as well as possible perivascular infiltration by lymphocytes and subepidermal appearance of elements similar to bubbles.
Clinical analysis will help to identify non-specific signs of inflammation, and a procedure such as a coagulogram will help to detect clotting disorders. With the help of a biochemical blood test, a reduced level of proteins is determined. The most valuable in the diagnosis of Stevens-Johnson syndrome is considered to be an immunological blood test. With the help of such a study, it is possible to detect an increased level of T-lymphocytes, as well as specific antibodies.
To diagnose Stevens-Johnson syndrome, you will need:
implementation of back-seeding of separable elements;
coprogram;
urine analysis (biochemical);
taking a sample of Zimnitsky;
CT of the kidneys;
Ultrasound of the kidneys and bladder;
lung X-ray.
If necessary, the patient can seek advice from a urologist, pulmonologist, oculist and nephrologist.
Differential diagnostics is also carried out in order to distinguish Stevens-Johnson syndrome from dermatitis, which, as is known, is characterized by the presence of blisters (simple contact dermatitis, During’s herpetiform dermatitis, allergic dermatitis, various forms of pemphigus, including vulgar, true, leaf-like and vegetative, as well as Lyell’s syndrome).
Treatment of Stevens-Johnson syndrome
Stevens-Johnson syndrome is treated with significant doses of glucocorticoid hormones. Due to the lesion of the oral mucosa, drugs have to be injected. Gradually, the doctor begins to lower the administered doses only after the symptoms become less pronounced, when the patient’s condition improves.
In order to purify the blood from the immune complexes formed in Stevens-Johnson syndrome, the method of extracorporeal hemocorrection is usually used, including membrane plasmapheresis, immunosorption, cascade filtration and hemosorption. Specialists carry out transfusion of plasma and protein solution. Great importance is given to the amount of fluid injected into the human body, as well as the maintenance of daily diuresis. For the purpose of additional therapy, drugs containing calcium and potassium are used. If we talk about the treatment and prevention of secondary infection, then it is carried out with the help of local or systemic antibacterial drugs.
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