The most severe allergic reaction - Stevens-Johnson syndrome: what it is and how to treat the disease. Stevens Johnson syndrome Stevens Johnson syndrome in children

Stevens-Johnson syndrome refers to severe systemic delayed-type allergic reactions (immune complex) and is a severe variant of erythema multiforme ( ), in which, along with skin damage, there is damage to the mucous membranes of at least two organs.

The causes of Stevens-Johnson syndrome are divided into four categories.

• Medicines. An acute toxic-allergic reaction occurs in response to the administration of a therapeutic dose of a drug. The most common causally significant drugs: antibiotics (especially penicillin) - up to 55%, non-steroidal anti-inflammatory drugs - up to 25%, sulfonamides - up to 10%, vitamins and other drugs that affect metabolism - up to 8%, local anesthetics - up to 6 %, other groups of medications (anti-epileptic drugs (carbamazepine), barbiturates, vaccines, and heroin) - up to 18%.
• Infectious agents. An infectious-allergic form is distinguished in association with viruses (herpes, AIDS, influenza, hepatitis, etc.), mycoplasmas, rickettsia, various bacterial pathogens (group A b-hemolytic streptococcus, diphtheria, mycobacteria, etc.), fungal and protozoal infections.
• Oncological diseases.
• Idiopathic Stevens-Johnson syndrome is diagnosed in 25-50% of cases.

Clinical picture

Stevens-Johnson syndrome most often occurs at the age of 20-40 years, but cases of its development have also been described in three-month-old children. Men get sick 2 times more often than women. As a rule (in 85% of cases), the disease begins with manifestations of an upper respiratory tract infection. The prodromal flu-like period lasts from 1 to 14 days and is characterized by fever, general weakness, cough, sore throat, headache, and arthralgia. Vomiting and diarrhea are sometimes observed. Damage to the skin and mucous membranes develops rapidly, usually after 4-6 days, can be localized anywhere, but symmetrical rashes are more typical on the extensor surfaces of the forearms, legs, dorsum of the hands and feet, face, genitals, and mucous membranes. Edema, clearly demarcated, flattened papules of pink-red color, round in shape, with a diameter of several millimeters to 2-5 cm, appear, having two zones: internal (grayish-bluish in color, sometimes with a bubble in the center filled with serous or hemorrhagic contents) and external (Red). Diffuse erythema, blisters, and erosive areas covered with a yellowish-gray coating appear on the lips, cheeks, and palate. After opening large blisters, continuous bleeding painful lesions form on the skin and mucous membranes, while the lips and gums become swollen, painful, with hemorrhagic crusts (Fig. 2, 3). The rash is accompanied by burning and itching. Erosive damage to the mucous membranes of the genitourinary system can be complicated by urethral strictures in men, bleeding from the bladder and vulvovaginitis in women. When the eyes are affected, blepharoconjunctivitis and iridocyclitis are observed, which can lead to loss of vision. Bronchiolitis, colitis, and proctitis rarely develop. Common symptoms include fever, headache and joint pain.

Prognostically unfavorable factors for Stevens-Johnson syndrome and Lyell's syndrome include: age over 40 years, rapidly progressing course, tachycardia with heart rate (HR) more than 120 beats/min, initial area of ​​epidermal lesion more than 10%, hyperglycemia more than 14 mmol /l.

The mortality rate for Stevens-Johnson syndrome is 3-15%. When the mucous membranes of internal organs are damaged, stenosis of the esophagus and narrowing of the urinary tract can form. Blindness due to secondary severe keratitis is recorded in 3-10% of patients.

Differential diagnosis should be made between erythema multiforme, Stevens-Johnson syndrome and Lyell's syndrome ( ). It should be remembered that similar skin lesions can occur with primary systemic vasculitis (hemorrhagic vasculitis, polyarteritis nodosa, microscopic polyarteritis, etc.).

Diagnostics

When collecting anamnesis, the patient should be asked the following questions:

• Has he had any allergic reactions before? What caused them? How did they manifest themselves?
• What preceded the development of an allergic reaction this time?
• What medications did the patient use the day before?
• Were the rashes preceded by symptoms of a respiratory infection (fever, general weakness, headache, sore throat, cough, arthralgia)?
• What measures did the patient take independently and how effective were they?

It is mandatory to record the presence of drug allergies in the medical documentation.

During the initial examination, pay attention to the presence of changes in the skin and visible mucous membranes, note the nature of the rash, localization, indicate the percentage of skin damage, the presence of blisters, epidermal necrosis; stridor, dyspnea, wheezing, dyspnea, or apnea; hypotension or a sharp decrease in normal blood pressure (BP); gastrointestinal symptoms (nausea, abdominal pain, diarrhea); pain when swallowing, urinating; change of consciousness.

An objective examination includes measurement of heart rate, blood pressure, body temperature, palpation examination of the lymph nodes and abdominal cavity.

Laboratory research:

• Detailed general blood test daily - until the condition stabilizes.
• Biochemical blood test: glucose, urea, creatinine, total protein, bilirubin, aspartate aminotransferase (AST), alanine aminotransferase (ALT), C-reactive protein (CRP), fibrinogen, acid-base status (ALS).
• Coagulogram.
• General urine test daily until the condition stabilizes.
• Cultures from the skin and mucous membranes, bacteriological examination of sputum, feces - according to indications.

In order to verify skin rashes and damage to mucous membranes, a consultation with a dermatologist is indicated. If there are signs of damage to other organs and systems, it is advisable to consult other specialists (otolaryngologist, ophthalmologist, urologist, etc.).

Emergency care at the prehospital stage

With the development of Stevens-Johnson syndrome, the main direction of emergency treatment is to replace fluid loss, as in burn patients (even if the patient’s condition is stable at the time of examination). Catheterization of the peripheral vein is carried out and fluid transfusion begins (colloid and saline solutions 1-2 l), if possible, oral rehydration.

Intravenous jet administration of glucocorticosteroids is used (in terms of intravenous prednisolone 60-150 mg). However, the effectiveness of prescribing systemic hormones is questionable. It is considered advisable to use pulse therapy in high doses in the early stages from the onset of an acute toxic-allergic reaction, because their planned administration increases the risk of septic complications and can lead to an increase in the number of deaths.

There must be readiness for artificial pulmonary ventilation (ALV), tracheotomy if asphyxia develops and immediate hospitalization in the intensive care unit.

Principles of inpatient therapy

The main measures are aimed at correcting hypovolemia, carrying out nonspecific detoxification, preventing the development of complications, primarily infections, as well as eliminating repeated exposure to the allergen.

It is necessary to discontinue all medications, except for those that are necessary for the patient for health reasons.

Appointed:

A general nonspecific hypoallergenic diet according to A.D. Ado is prescribed as a hypoallergenic diet. It involves excluding the following foods from the diet: citrus fruits (oranges, tangerines, lemons, grapefruits, etc.); nuts (peanuts, hazelnuts, almonds, etc.); fish and fish products (fresh and salted fish, fish broths, canned fish, caviar, etc.); poultry meat (goose, duck, turkey, chicken, etc.) and products made from it; chocolate and chocolate products; coffee; smoked products; vinegar, mustard, mayonnaise and other spices; horseradish, radish, radish; tomatoes, eggplants; mushrooms; eggs; unleavened milk; strawberry, melon, pineapple; butter dough; honey; alcoholic drinks.

Can be used:

• lean beef meat, boiled;
• soups: cereal, vegetable:

  With recycled beef broth;

  Vegetarian with the addition of butter, olive, sunflower oil;

• Boiled potatoes;
• porridge: buckwheat, rolled oats, rice;
• one-day lactic acid products (cottage cheese, kefir, yogurt);
• fresh cucumbers, parsley, dill;
• baked apples, watermelon;
• sugar;
• compotes from apples, plums, currants, cherries, dried fruits;
• white unsweetened bread.

The diet includes about 2800 kcal (15 g protein, 200 g carbohydrates, 150 g fat).

Possible complications:

• Ophthalmological - corneal erosions, anterior uveitis, severe keratitis, blindness.
• Gastroenterological - colitis, proctitis, esophageal stenosis.
• Urogenital - tubular necrosis, acute renal failure, bleeding from the bladder, urethral stricture in men, vulvovaginitis and vaginal stenosis in women.
• Pulmonological - bronchiolitis and respiratory failure.
• Skin - scars and cosmetic defects that arise during healing and the addition of a secondary infection.

Typical mistakes:

The use of low doses of glucocorticosteroids at the onset of the disease and long-term glucocorticosteroid therapy after stabilization of the patient's condition;

Prophylactic administration of antibacterial drugs in the absence of infectious complications.

Let us emphasize once again that penicillin drugs are strictly contraindicated and the administration of vitamins (group B, ascorbic acid, etc.) is contraindicated, since they are strong allergens.

The use of calcium preparations (calcium gluconate, calcium chloride) is pathogenetically unjustified and can unpredictably affect the further course of the disease.

The patient is constantly reminded that medications should be taken only as prescribed by the doctor. The patient is given a reminder about drug intolerance, referred for a consultation with an allergist or clinical immunologist, and is recommended to study at an allergy school. The patient is taught the correct use of emergency medications, injection techniques in case of repeated contact with the allergen and the occurrence of severe acute toxic-allergic reactions (anaphylactic shock). Your home medicine cabinet should include adrenaline, glucocorticosteroids (prednisolone) for parenteral administration, syringes, needles and antihistamines.

Prevention of the development of drug allergies includes compliance with the following rules.

• A thorough collection and analysis of pharmacological history should be carried out.
• The title page of the outpatient and/or inpatient chart should indicate the drug that caused the allergy, the reaction, its type and the date of the reaction.
• You cannot prescribe a drug (and combination drugs containing it) that previously caused an allergic reaction.
• You should not prescribe a drug that belongs to the same chemical group as an allergen drug, taking into account the possibility of developing cross-allergy.
• It is necessary to avoid prescribing multiple medications at the same time.
• The instructions for administering the drug should be strictly followed.
• Prescribe doses of medications in accordance with the age, body weight of the patient and taking into account concomitant pathology.
• It is not recommended to prescribe drugs with histaminoliberation properties (paracetamol, valpromide, valproic acid, phenothiazine neuroleptics, pyrazolone drugs, gold salts, etc.) to patients suffering from diseases of the gastrointestinal tract, hepatobiliary system and metabolism.
• If there is a need for emergency surgery, tooth extraction, or the introduction of radiopaque agents to persons with a history of drug allergies and if it is impossible to clarify the nature of the existing adverse reactions, premedication should be carried out: 1 hour before the intervention - intravenous drip of glucocorticosteroids (4-8 mg of dexamethasone or 30-60 mg prednisolone) in saline and antihistamines.

Thus, Stevens-Johnson syndrome is a serious disease that requires early diagnosis, hospitalization of the patient, careful care and observation, and rational drug therapy.

Literature

1. Clinical recommendations. Patient management standards. M.: GEOTAR Medicine, 2005. 928 p.
2. Hurwitz S. Erythema multiforme: a review of its characteristics, diagnostic criteria, and management//Pediatr. Rev. 1990; 11: 7: 217-222.
3. Hallgren J., Tengvall-Linder M., Persson M. Stevens-Johnson syndrome associated with ciprofloxacin: A review of adverse cutaneous events reported in Sweden as associated with this drug//J. Amer. Acad. Derm. 2003; 49:5.
4. Hofbauer G. F., Burg G., Nestle F. O. Cocaine-related Stevens-Johnson syndrome//Dermatology. 2000; 201:3:258-260.
5. Parrillo S. J. Stevens-Johnson Syndrome//eMedicine, December 2004. — http://www.emedicine.com/
6. Bastuji-Garin S., Fouchard N., Bertocchi M., Roujeau J. C., Revuz J., Wolkenstein P. SCORTEN: a severity-of-illness score for toxic epidermal necrolysis // J. Invest Dermatol. 2000; 115(2): 149-153.
7. Shear N., Esmail N., Ing S., Kerba M. Dermatology/Review Notes and Lecture Series//MCCQE, 2000; 21.
8. Drannik G. N. Clinical immunology and allergology. M.: Medical Information Agency LLC, 2003. 604 p.
9. Vertkin A. L. Emergency medical care. Guide for paramedics: textbook. allowance. M.: GEOTAR Medicine, 2005. 400 p.
10. Immunopathology and allergology. Algorithms for diagnosis and treatment / ed. R. M. Khaitova. M.: GEOTAR Medicine, 2003. 112 p.

A. L. Vertkin, Doctor of Medical Sciences, Professor
A. V. Dadykina, Candidate of Medical Sciences
NNPOS MP, MGMSU, TsPK and PPS Nizhny Novgorod State Medical Academy, Moscow, Nizhny Novgorod

• Which doctors should you see if you have Stevens-Johnson Syndrome?

What is Stevens-Johnson Syndrome

Stevens-Johnson syndrome(malignant exudative erythema) is a very severe form of erythema multiforme, in which blisters appear on the mucous membrane of the mouth, throat, eyes, genitals, and other areas of the skin and mucous membranes.

Damage to the oral mucosa makes it difficult to eat; closing the mouth causes severe pain, which leads to drooling. The eyes become very painful, swollen and filled with pus so that the eyelids sometimes stick together. The corneas undergo fibrosis. Urination becomes difficult and painful.

What Causes Stevens-Johnson Syndrome?

The main reason for the occurrence Stevens-Johnson syndrome is the development of an allergic reaction in response to taking antibiotics and other antibacterial drugs. Currently, a hereditary mechanism for the development of pathology is considered very likely. As a result of genetic disorders in the body, its natural defenses are suppressed. In this case, not only the skin itself is affected, but also the blood vessels that feed it. It is these facts that determine all the developing clinical manifestations of the disease.

Pathogenesis (what happens?) during Stevens-Johnson Syndrome

The disease is based on intoxication of the patient’s body and the development of allergic reactions in it. Some researchers tend to consider pathology as a malignant type of multimorphic exudative erythema.

Symptoms of Stevens-Johnson Syndrome

This pathology always develops in the patient very quickly, rapidly, since in essence it is an immediate allergic reaction. Initially, severe fever and pain in the joints and muscles appear. Subsequently, after just a few hours or a day, damage to the oral mucosa is detected. Here bubbles of quite large sizes appear, skin defects covered with gray-white films, crusts consisting of clots of dried blood, and cracks.

Defects also appear in the area of ​​the red border of the lips. Eye damage occurs as conjunctivitis (inflammation of the mucous membranes of the eyes), but the inflammatory process here is purely allergic in nature. In the future, bacterial damage may also occur, as a result of which the disease begins to progress more severely, and the patient’s condition sharply worsens. Small defects and ulcers may also appear on the conjunctiva with Stevens-Johnson syndrome, and inflammation of the cornea and posterior parts of the eye (retina vessels, etc.) may occur.

Lesions can often also involve the genitals, which manifests itself in the form of urethritis (inflammation of the urethra), balanitis, vulvovaginitis (inflammation of the female external genitalia). Sometimes mucous membranes in other places are involved. As a result of skin damage, a large number of red spots are formed on it with raised areas above the skin level, like blisters. They have rounded outlines and a purple color. In the center they are bluish and seem to be somewhat sunken. The diameter of the lesions can range from 1 to 3-5 cm. In the central part of many of them, blisters form, which contain a transparent watery liquid or blood inside.

After opening the blisters, bright red skin defects remain in their place, which are then covered with crusts. Mostly, the lesions are located on the patient’s torso and in the perineal area. There is a very pronounced disturbance in the general condition of the patient, which manifests itself in the form of severe fever, malaise, weakness, fatigue, headache, and dizziness. All these manifestations last on average about 2-3 weeks. Complications during the disease may include pneumonia, diarrhea, kidney failure, etc. In 10% of all patients, these diseases are very severe and lead to death.

Diagnosis of Stevens-Johnson Syndrome

When conducting a general blood test, an increased content of leukocytes, the appearance of their young forms and specific cells responsible for the development of allergic reactions, and an increase in the erythrocyte sedimentation rate are detected. These manifestations are very nonspecific and occur in almost all inflammatory diseases. A biochemical blood test may detect an increase in the content of bilirubin, urea, and aminotransferase enzymes.

The clotting ability of blood plasma is impaired. This is due to a decrease in the content of the protein responsible for coagulation - fibrin, which, in turn, is a consequence of an increase in the content of enzymes that carry out its breakdown. The total protein content in the blood also becomes significantly reduced. The most informative and valuable in this case is to conduct a specific study - an immunogram, during which a high content of T-lymphocytes and certain specific classes of antibodies in the blood is detected.

To make a correct diagnosis of Stevens-Johnson syndrome, it is necessary to interview the patient as fully as possible about his living conditions, diet, medications taken, working conditions, diseases, especially allergic ones, of his parents and other relatives. The time of onset of the disease, the effect on the body of various factors that preceded it, especially the use of medications, are clarified in detail. The external manifestations of the disease are assessed, for which the patient must be undressed and the skin and mucous membranes carefully examined. Sometimes it is necessary to distinguish the disease from pemphigus, Lyell's syndrome and others, but in general, making a diagnosis is a fairly simple task.

Treatment of Stevens-Johnson Syndrome

Preparations of adrenal hormones in medium dosages are mainly used. They are administered to the patient until there is a lasting significant improvement in the condition. Then the dosage of the drug begins to be gradually reduced, and after 3-4 weeks it is completely discontinued. In some patients, the condition is so severe that they are unable to take medications by mouth on their own. In these cases, hormones are administered in liquid forms intravenously. Very important are procedures that are aimed at removing immune complexes, which are antibodies associated with antigens, from the body circulating in the blood. For this purpose, special drugs for intravenous administration and blood purification methods in the form of hemosorption and plasmapheresis are used.

Drugs taken orally are also used to help remove toxic substances from the body through the intestines. In order to combat intoxication, at least 2-3 liters of liquid should be introduced into the patient’s body daily through various routes. At the same time, make sure that this entire volume is removed from the body in a timely manner, since when fluid is retained, toxins are not washed out and quite severe complications can develop. It is clear that the full implementation of these measures is only possible in an intensive care unit.

A fairly effective measure is intravenous transfusion of solutions of proteins and human plasma to the patient. Additionally, medications containing calcium, potassium, and antiallergic drugs are prescribed. If the lesions are very large and the patient’s condition is quite severe, then there is always a risk of developing infectious complications, which can be prevented by prescribing antibacterial agents in combination with antifungal drugs. In order to treat skin rashes, various creams containing preparations of adrenal hormones are applied topically. To prevent infection, various antiseptic solutions are used.

Forecast

As already mentioned, 10% of all patients with Stevens-Johnson syndrome die as a result of severe complications. In other cases, the prognosis of the disease is quite favorable. Everything is determined by the severity of the disease itself, the presence of certain complications.

The name DDS (Lyell and Johnson syndrome) refers to severe inflammation of the skin and mucous membranes - erythema multiforme. The disease was discovered in the USA in 1922. It was described by two pediatricians, Lyell and Johnson, after whom the syndrome got its name. The disease is characterized by an acute course, with damage to the mucous membranes and skin of at least two organs.
Currently, Lyell and Stevens Johnson syndrome occurs throughout the world in people mainly over the age of forty. Recently, the disease has begun to be diagnosed in children and even infants.

Prerequisites for the disease:

1. Taking certain medications;
Often, signs of Lyell and Stevens Johnson syndrome occur with an overdose of drugs. There are cases of the disease due to taking just one and the correct dose of medicine. Most cases of the disease were provoked by taking antibiotics, more often when taking antibiotics from the penicillin series.
There is a lower likelihood of the disease when taking local anesthetics, sulfa drugs, and non-steroidal anti-inflammatory drugs.
2. Infectious diseases;
Increased risk factors are bacterial and fungal infections. An acute allergic reaction to SSD can develop after contact with herpes, HIV, influenza and hepatitis viruses.
3. Oncological diseases;
4. Idiopathic form.
This is a disease whose causes experts have not been able to identify. Lyell-Stevens Johnson syndrome may occur for an unknown reason.

Main symptoms of SSD

In most cases, the syndrome begins to manifest as severe pain in the joints. SSD is an acute or rapid form of allergy and therefore begins unexpectedly.
The patient may initially think that he is suffering from a respiratory disease. At this stage, severe persistent weakness, joint pain, and fever are felt. An ill person may suffer from nausea and vomiting. The initial state of the syndrome lasts several hours or several days. Then severe rashes appear on the mucous membranes and skin.
The rash appears in different places. A feature of the syndrome is symmetrical rashes. An allergic reaction makes itself felt with severe itching and a burning sensation.
The rash is localized in different ways. Most patients experience rashes on the face, back of the hands and feet, and on the bends of the knees and elbows. The mucous membranes in the mouth are most often affected, and a little less often - the eyes.
Lyell and Stevens Johnson syndrome is characterized by a papule-shaped rash with a diameter of two to five millimeters. Each papule is visually divided into two parts. In the center of the vesicle, a small cavity with blood (hemorrhagic contents) and protein fluid (serous substance) is clearly visible. The outer part of the bubble is bright red.
Papules that form on the mucous membranes are more painful. They quickly burst, leaving painful erosions with an unhealthy yellow coating in place.
Damage to the mucous membranes in intimate places can lead to urethral stricture in men and to the development of vaginitis in women.
When the eyes are damaged, blepharoconjunctivitis and other eye diseases that impair vision develop.
With any form of Lyell and Stevens Johnson syndrome, patients experience pain and an increasing feeling of anxiety. Pain in the rash on the mucous membranes leads to refusal of food.

Diagnosis of SSc syndrome

A detailed and correct anamnesis must be compiled. SSD is one of the severe forms of an allergic reaction, so it is possible to identify some patterns in the early allergic manifestations of a sick person. The patient must provide the attending physician with the most complete information possible - tell whether allergic reactions have occurred before. Also, the doctor must know which substances and how severe the allergy was.
The attending physician prescribes a general blood test and biochemical study for the patient. The test results will reveal aminotransferase enzymes, bilirubin and urea in the blood.
Through a thorough external examination, the strength and nature of the allergic lesion is revealed. Often, a correct diagnosis is impossible without an immunogram. This study is aimed at searching for antibodies of a specific class in the blood.
Diagnosing Lyell and Stevens Johnson syndrome itself is not an extremely difficult task. Almost all prescribed studies are aimed at excluding diseases such as pemphigus or other diseases with similar clinical manifestations.
How is SSD treated?
Detection of Lyell and Stevens Johnson syndrome requires urgent qualified medical assistance.
What urgent needs does the patient need before being admitted to the inpatient department? The following urgent measures are being taken:
Vein catheterization;
Infusion therapy (saline or colloid solutions are administered to reduce the concentration of allergens in the blood);
Intravenous administration of prednisolone in a single dose of 60-150 mg;
Due to severe swelling of the laryngeal mucosa, the patient is transferred to a mechanical ventilation device.
After the patient is admitted and his condition is stabilized in the hospital department, he is prescribed basic treatment. The attending physician will prescribe analgesics for pain relief and glucocorticosteroid drugs against inflammation. If the skin or mucous membranes are infected, strong antibiotics are prescribed, except for drugs based on penicillin and vitamins.
Complications accompanying Lyell and Stevens Johnson syndrome require additional therapy.
Lesions of the skin and mucous membranes are treated with antiseptic solutions and anti-inflammatory ointments.
The patient should receive a hypoallergenic diet, excluding the consumption of all types of fish, coffee, citrus fruits, honey and chocolate.

Stevens-Johnson syndrome is an acute bullous lesion of the mucous membranes and skin. People aged 20–40 years are susceptible to the disease; it is extremely rarely diagnosed in infants under 3 years of age. The pathology is observed mainly in men. The syndrome is characterized by an acute course and rapid development of complications with damage to internal organs. This determines the need for prompt provision of qualified assistance.

Causes

Doctors say the main reason for the development of Stevens-Johnson syndrome is taking medications. An acute allergic reaction occurs with an overdose of drugs or in case of individual intolerance to the components. As a rule, these are antibiotics of the penicillin group, non-steroidal anti-inflammatory drugs, central nervous system regulators, painkillers, sulfonamides and vitamins.

Less commonly, Stevens-Johnson syndrome is caused by infectious diseases. The infectious-allergic form occurs when affected by herpes, influenza, hepatitis or HIV, and in childhood the causative agent is measles, mumps and chickenpox viruses. Sometimes a negative reaction is possible with fungal and bacterial infections.

The syndrome can be triggered by an oncological disease (carcinoma or lymphoma). Sometimes doctors are unable to establish the etiology of the disease, in which case they speak of the idiopathic form.

Symptoms

Stevens-Johnson syndrome is a fulminant allergic reaction that develops rapidly and is very acute. The first symptoms are similar to those of a respiratory disease. The patient develops weakness, fever, temperature rise up to 40 ⁰C, joint pain, headache and drowsiness. A sore or sore throat or dry cough may appear.

In some cases, dyspeptic disorders are observed: nausea, vomiting, diarrhea and complete lack of appetite. Disorders of the cardiovascular system - tachycardia (rapid heartbeat) and increased heart rate.

This condition persists for several hours, and then a symptom characteristic of the syndrome appears - a rash on the skin and mucous membranes.

The rash can be localized in different parts of the body, but, as a rule, the rashes are symmetrical. Mostly the allergic reaction is observed on the knee and elbow bends, on the face and on the back of the hand and feet. The rash also occurs on the mucous membranes - in the mouth, eyes and genitals. The rash is accompanied by severe burning and itching.

Externally, the rash looks like papules with a diameter of 2–4 mm. In the center of the formation there is a vesicle with serous or hemorrhagic fluid. The outer part of the papule is bright red. Bubbles localized on the mucous membranes quickly burst, leaving painful erosions in this place, which over time become covered with a yellow coating.

Damage to the mucous membrane of the eyes is similar to allergic conjunctivitis. A secondary infection often occurs, which causes an acute inflammatory process with purulent discharge. Erosive and ulcerative lesions form on the cornea and conjunctiva. Keratitis, blepharitis or iridocyclitis may develop.

If the oral mucosa and red border of the lips are affected, the patient experiences difficulty eating and drinking. Nutrition is provided through a tube, and medications are administered intravenously.

The patient's psycho-emotional state worsens. He experiences anxiety and irritability, becomes withdrawn and apathetic. Due to constant itching and pain, sleep is disturbed, appetite worsens and performance decreases.

Diagnostics

To diagnose the syndrome, anamnesis is taken. The doctor finds out whether the patient has a tendency to have an allergic reaction, whether it has happened before and what caused it. It is important to detect the fact of taking medications or the presence of an infectious process. The doctor conducts a visual examination, assessing the condition of the skin and mucous membranes.

Laboratory diagnostic methods: general and biochemical blood tests. The level of urea, bilirubin and aminotransferase enzymes is of diagnostic importance.

A coagulogram allows you to evaluate blood clotting and the rate of blood clot formation. An immunogram may be performed to detect specific antibodies in the blood. The presence of pathology is indicated by an increased level of T-lymphocytes.

Sometimes histological examination reveals necrosis of epidermal cells, and perivascular infiltration with lymphocytes is diagnosed.

Instrumental diagnostic methods: CT scan of the kidneys, radiography of the lungs, ultrasound of the urinary system. In some cases, additional consultation with a nephrologist, pulmonologist, urologist and ophthalmologist is required.

During diagnosis, it is important to differentiate Stevens-Johnson syndrome from pemphigus, Lyell's syndrome and other pathologies that have similar symptoms.

Treatment

Stevens-Johnson syndrome requires immediate medical attention. Before admitting the patient to the hospital, it is important to catheterize the vein and begin infusion therapy. To reduce the level of allergens in the blood, droppers with saline or colloidal solution are used. Additionally, the patient is given intravenous Prednisolone (60–150 mg). If swelling of the laryngeal mucosa develops and breathing becomes impaired, the patient is transferred to artificial ventilation.

After the acute attack subsides, the patient is placed in a hospital setting, where he is constantly under the supervision of medical personnel. Analgesics are prescribed to relieve pain and alleviate the condition. Glucocorticosteroids will help eliminate inflammation.

If necessary, intravenous transfusion of plasma and protein solution is performed. Additionally, medications high in calcium and potassium are prescribed. To combat allergies, antihistamines are prescribed - Suprastin, Diazolin or Loratadine.

In case of bacterial damage to the body, antibacterial therapy is carried out. At the same time, it is strictly forbidden to use antibiotics of the penicillin group and vitamin complexes. To improve the condition of the skin, anti-inflammatory ointments and antiseptic solutions are prescribed.

Prognosis and prevention

With timely assistance, the prognosis is quite favorable. However, the syndrome is often accompanied by severe complications, which makes treatment difficult. Typically, this is vaginitis in women and urethral stricture in men. When the mucous membranes of the eyes are damaged, blepharoconjunctivitis develops and visual acuity decreases. As a complication, the development of pneumonia, colitis, bronchiolitis and secondary infection is possible. Acute renal failure develops less frequently and the process of hormone production by the adrenal glands is disrupted. In 10% of cases, patients with Stevens-Johnson syndrome die.

Stevens Johnson syndrome is a very serious disease of a systematic delayed-type allergic reaction that occurs as exudative erythema multiforme, affecting least of all the mucous membrane of two organs, maybe more.

Causes

The causes of Steven Johnson syndrome can be divided into subgroups:

• medications. An acute allergic reaction occurs when a medication enters the body. The main groups causing Steven Johnson syndrome: penicillin antibiotics, non-steroidal anti-inflammatory drugs, sulfonamides, vitamins, barbiturates, heroin;
• infections. In this case, the infectious-allergic form of Steven Johnson syndrome is recorded. Allergens are: viruses, mycoplasmas, bacteria;
• oncological diseases;
• idiopathic form Stevens Johnson syndrome. In such a situation, clear reasons cannot be determined.

Clinical picture

Steven Johnson syndrome appears at a young age from 20 to 40 years, but there are times when this disease is diagnosed in newborn children. Men are more often affected than women.

The first symptoms affect the upper respiratory tract system by infection. The initial prodromal period extends to two weeks and is characterized by fever, severe weakness, cough, and headaches. In rare cases, vomiting and diarrhea are caused.

The skin and mucous membranes of the mouth in children and adults are affected instantly within five days; the location can be anywhere, but most often a rash occurs on the elbows, knees, face, reproductive organs and all mucous membranes.

With Steven Johnson syndrome, swollen, compacted papules of a dark pink color appear, round in shape, the diameter of which is from one to six centimeters. There are two zones: internal and external. The internal ones are characterized by a grayish-blue color, in the middle a bubble appears inside which contains serous fluid. The outer one appears in red.

In the oral cavity, on the lips, cheeks in children and adults, Stevens John syndrome is manifested by broken erythema, blisters, and erosive areas of a yellowish-gray color. When the blisters open, bleeding wounds form; lips and gums swell, hurt, and become covered with hemorrhagic crusts. The rash on all areas of the skin feels burning and itching.

In the urine excretory system, the mucous membranes are affected and is manifested by bleeding from the urine excretory tract, a complication of the urethra in men, and vulvovaginitis in girls. The eyes are also affected, in which case blephoroconjunctivitis progresses, which often leads to complete blindness. Rarely, but the development of colitis and proctitis is possible.

There are also general symptoms: fever, headaches and joint pain. Malignant exudative erythema develops in people over forty years of age, acute and very rapid, heart contractions become frequent, hyperglycemia. Symptoms of damage to internal organs, namely their mucous membranes, manifest themselves in the form of stenosis of the esophagus.

The final fatality rate for Steven Johnson syndrome is ten percent. Complete loss of vision after severe keratitis caused by Stephen John syndrome is observed in five to ten patients.

Exudative erythema multiforme is diagnosed together with Lyell's syndrome. It is held between them. In both of these diseases the primary lesions are similar. They may also be similar to systemic vasculitis.

Video: The Scary Reality About Stevens-Johnson Syndrome