Stevens Johnson syndrome causes. Stevens Johnson syndrome: is the disease as terrible as it looks. Complications and prognosis

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

The reasons

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

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

Clinical picture

Steven Johnson syndrome appears at a young age of 20 to 40 years, but there are times when such a disease is diagnosed in newborns. More often men are ill than women.

The first symptoms affect the infection of the upper respiratory tract. The initial prodromal period is extended to two weeks and is systematized by fever, severe weakness, cough, and headaches appear. In rare cases, vomiting, diarrhea is caused.

The skin and mucous membranes of the oral cavity in children and adults are affected instantly within five days, the location can be anything, but most often there is a rash on the elbows, knees, face, organs of the reproductive system and all mucous membranes.

With Steven Johnson syndrome, edematous, 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 one is characterized by a grayish-blue color, in the middle a bubble appears inside which contains a serous fluid. The outer appears in red.

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

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

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

The final lethal outcome in Steven Johnson syndrome is noted in ten percent. Complete loss of vision after severe keratitis caused by Steven John syndrome occurs in five to ten patients.

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

Video: The terrible reality of Stevens-Johnson syndrome

This is an acute bullous lesion of the mucous membranes and skin of an allergic nature. It proceeds against the background of a serious condition of the diseased with the involvement of the oral mucosa, eyes and urinary organs. Diagnosis of Stevens-Johnson syndrome includes a thorough examination of the patient, an immunological blood test, a skin biopsy, and a coagulogram. According to the indications, X-rays of the lungs, ultrasound of the bladder, ultrasound of the kidneys, biochemical analysis of urine, consultations of other specialists are performed. Treatment is carried out by methods of extracorporeal hemocorrection, glucocorticoid and infusion therapy, antibacterial drugs.

ICD-10

L51.1 Bullous erythema multiforme

General information

Data on Stevens-Johnson syndrome were published in 1922. Over time, the syndrome was named after the authors who first described it. The disease is a severe variant of exudative erythema multiforme and has a second name - "malignant exudative erythema". Together with Lyell's syndrome, pemphigus, bullous variant of SLE, allergic contact dermatitis, Hailey-Hailey disease, etc., clinical dermatology classifies Stevens-Johnson syndrome as bullous dermatitis, the common clinical symptom of which is the formation of blisters on the skin and mucous membranes.

Stevens-Johnson syndrome occurs at any age, most often in persons 20-40 years old and extremely rare in the first 3 years of a child's life. According to various data, the prevalence of the syndrome per 1 million population ranges from 0.4 to 6 cases per year. Most authors note a higher incidence among men.

The reasons

The development of Stevens-Johnson syndrome is due to an allergic reaction of an immediate type. There are 4 groups of factors that can provoke the onset of the disease: infectious agents, drugs, malignant diseases and unknown causes.

In childhood, Stevens-Johnson syndrome often occurs against the background of viral diseases: herpes simplex, viral hepatitis, adenovirus infection, measles, influenza, chicken pox, mumps. The provoking factor can be bacterial (salmonellosis, tuberculosis, yersiniosis, gonorrhea, mycoplasmosis, tularemia, brucellosis) and fungal (coccidioidomycosis, histoplasmosis, trichophytosis) infections.

In adults, Stevens-Johnson syndrome is usually due to medication or malignancy. Of the drugs, the role of the causative factor is primarily assigned to antibiotics, non-steroidal anti-inflammatory drugs, CNS regulators and sulfonamides. The leading role among oncological diseases in the development of Stevens-Johnson syndrome is played by lymphomas and carcinomas. If a specific etiological factor of the disease cannot be established, then they speak of the idiopathic Stevens-Johnson syndrome.

Symptoms

Stevens-Johnson syndrome is characterized by an acute onset with rapid development of symptoms. At the beginning, there is malaise, a rise in temperature to 40 ° C, headache, tachycardia, arthralgia and muscle pain. The patient may experience sore throat, cough, diarrhea, and vomiting. After a few hours (maximum after a day), rather large blisters appear on the oral mucosa. After their opening, extensive defects are formed on the mucosa, covered with white-gray or yellowish films and crusts of gore. The red border of the lips is involved in the pathological process. Due to severe mucosal damage in Stevens-Johnson syndrome, patients cannot eat or even drink.

Eye damage at the beginning proceeds according to the type of allergic conjunctivitis, but is often complicated by secondary infection with the development of purulent inflammation. For Stevens-Johnson syndrome, the formation of erosive-ulcerative elements of small size on the conjunctiva and cornea is typical. Possible damage to the iris, the development of blepharitis, iridocyclitis, keratitis.

The defeat of the mucous organs of the genitourinary system is observed in half of the cases of Stevens-Johnson syndrome. It proceeds in the form of urethritis, balanoposthitis, vulvitis, vaginitis. Scarring of erosions and ulcers of the mucosa can lead to the formation of urethral stricture.

The skin lesion is represented by a large number of rounded raised elements resembling blisters. They are purple in color and reach a size of 3-5 cm. A feature of the elements of the skin rash in Stevens-Johnson syndrome is the appearance of serous or bloody blisters in their center. The opening of the blisters leads to the formation of bright red defects, covered with crusts. The favorite localization of the rash is the skin of the trunk and perineum.

The period of appearance of new rashes of Stevens-Johnson syndrome lasts approximately 2-3 weeks, the healing of ulcers occurs within 1.5 months. The disease can be complicated by bleeding from the bladder, pneumonia, bronchiolitis, colitis, acute renal failure, secondary bacterial infection, loss of vision. As a result of developed complications, about 10% of patients with Stevens-Johnson syndrome die.

Diagnostics

The clinician-dermatologist can diagnose Stevens-Johnson syndrome based on the characteristic symptoms detected during a thorough dermatological examination. Questioning the patient allows you to determine the causative factor that caused the development of the disease. A skin biopsy helps confirm the diagnosis of Stevens-Johnson syndrome. Histological examination shows epidermal cell necrosis, perivascular lymphocyte infiltration, and subepidermal blistering.

In a clinical blood test, nonspecific signs of inflammation are determined, a coagulogram reveals clotting disorders, and a biochemical blood test shows a low protein content. The most valuable in terms of diagnosing Stevens-Johnson syndrome is an immunological blood test, which detects a significant increase in T-lymphocytes and specific antibodies.

Stevens-Johnson syndrome is a toxic-allergic disease, which is a malignant variant of bullous exudative erythema multiforme.

Etiology and pathogenesis. The reason for its development may be drugs (antibiotics, sulfonamides, analgesics, barbiturates, etc.). The development of the process is based on toxic-allergic reactions. A hereditary predisposition to the development of Stevens-Johnson syndrome is assumed, expressed in the suppression of natural resistance factors, T-cell immunodeficiency and an increase in peripheral blood of B-lymphocytes. The allergic reaction that develops to allergen exposure is directed to keratinocytes with the formation of circulating immune complexes in the blood serum, the deposition of IgM and the C3-component of complement along the basement membrane of the epidermis and in the superficial dermal blood vessels.

clinical picture. The disease develops acutely with high fever, arthralgia, myalgia. Then, after a few hours or 2-3 days, lesions of the mucous membranes of the oral cavity appear (formation of blisters, erosion with gray-white films, hemorrhagic crusts, cracks, erosion on the red border of the lips), eyes in the form of conjunctivitis with the formation of erosive-ulcerative areas, keratitis , uveitis, genital mucosa with the development of urethritis, balanitis, vulvovaginitis. Other mucous membranes may also be involved in the process. The skin lesion is characterized by disseminated erythemato-papular rounded elements with a purple-colored peripheral zone and a sunken bluish center with a diameter of 1 to 3-5 cm, in the center of many of them blisters with serous or hemorrhagic contents form.

Opening, the bubbles leave juicy bright red erosion, covered with crusts over time. The main localization of the skin lesion is on the trunk, in the perineum. Severe general phenomena (fever, malaise, headaches, etc.) continue for 2-3 weeks. Against this background, pneumonia, diarrhea, kidney failure and other complications can develop, which in 5-10% of cases lead to death. In the blood, leukocytosis with a shift of the formula to the left, eosinopenia, and an increase in ESR are noted. There may be an increase in bilirubin, urea, aminotransferases, changes in plasma fibrinolytic activity due to the activation of the proteolytic system, a decrease in the total amount of protein due to albumins. The immunogram shows a decrease in the total number of T-lymphocytes and immunoglobulins of classes G, M.

The diagnosis is based on the anamnesis, clinical picture and laboratory data. Differential diagnosis is carried out with pemphigus, Lyell's syndrome, etc.

Treatment: corticosteroid drugs in medium doses (30-60 mg per day orally in terms of prednisone) until a pronounced clinical effect is achieved, followed by a gradual dose reduction until complete withdrawal within 3-4 weeks. If oral administration is not possible, corticosteroids are prescribed parenterally. They also carry out activities aimed at inactivation and removal of antigens and circulating immune complexes from the body, using enterosorbents, hemosorption, plasmapheresis. To combat the syndrome of endogenous intoxication, 2-3 liters of liquid are administered parenterally per day (physiological saline, gemodez, Ringer's solution, etc.), as well as albumin, plasma. Calcium, potassium, antihistamines are also prescribed, in case of a threat of infection, broad-spectrum antibiotics. Outwardly, corticosteroid creams are used (Lorinden C, Celestoderm with Garomycin, etc.), 2% aqueous solutions of methylene blue, gentian violet.

Stevens-Johnson syndrome is a very severe skin disease, a malignant type of exudative erythema, in which severe redness appears on the skin. At the same time, large blisters appear on the mucous membranes and skin. Inflammation of the oral mucosa makes it difficult to close the mouth, eat, drink. Severe pain provokes increased salivation, difficulty breathing.

Inflammation, the appearance of blisters on the mucous membranes of the genitourinary system makes it difficult for natural administration. Urination and intercourse become very painful.

Most often, Stevens-Jones syndrome occurs as a response to an allergic reaction to antibiotics or drugs designed to treat bacterial infections. Representatives of medicine are inclined to believe that the tendency to the disease is inherited.

The cause of the exacerbation may be, according to scientists, several factors.

Most often, Stevens-Jones syndrome occurs as a response to an allergic reaction to antibiotics or drugs designed to treat bacterial infections. The reaction can cause drugs for epilepsy, sulfonamides, non-steroidal painkillers. Many drugs, especially those of synthetic origin, also contribute to the symptoms that characterize Stevenson Johnson syndrome.

Infectious diseases (flu, AIDS, herpes, hepatitis) can also provoke a malignant form of exudative erythema. Fungi, mycoplasmas, bacteria that enter the body can provoke an allergic reaction

Finally, symptoms are very often recorded in the presence of oncological diseases.

More often than others, Stevens Johnson syndrome manifests itself in men from twenty to forty years old, although the disease has been recorded in women, children up to six months.

Since the disease belongs to instant-type allergies, it develops very quickly. It begins with severe malaise, the appearance of unbearable pain in the joints, muscles, a sharp increase in temperature.

After a few hours (rarely - days), the skin is covered with silvery films, deep cracks, blood clots.

At this time, blisters appear on the lips and eyes. If initially an allergic reaction in the eyes comes down to their strong redness, then later ulcers and purulent blisters may appear. The cornea, the back of the eye becomes inflamed.

Stevens-Johnson syndrome can affect the genitals, causing cystitis or urethritis.

A complete blood count is required to make a diagnosis. Usually, in the presence of the disease, it shows a very high level of leukocytes, rapid erythrocyte sedimentation.

In addition to the general analysis, it is necessary to take into account all the drugs, substances, food that the patient took.

The treatment of the syndrome usually involves intravenous transfusion of blood plasma, drugs that cleanse the body of accumulated toxins, and the administration of hormones. To prevent the development of infection in ulcers, a complex of antifungal and antibacterial drugs, antiseptic solutions are prescribed.

It is very important to follow a strict diet prescribed by a doctor, drink plenty of fluids.

It has been statistically established that with a timely visit to a specialist, the treatment ends quite successfully, although it takes a long time. Therapy usually lasts 3-4 months.

If the sick person did not start receiving drug treatment in the first days of the disease, then Stevens-Johnson syndrome can be fatal. 10% of patients die due to late treatment.

Sometimes after treatment, especially if the disease was severe, scars or spots may remain on the skin. It is not excluded the appearance of complications in the form of colitis, respiratory failure, dysfunction of the genitourinary system, blindness.

This disease absolutely excludes self-treatment, as it is potentially fatal.

Among the seemingly harmless, there are also acute, one might even say, serious forms of diseases provoked by an allergen. These include Stevens Johnson Syndrome. It has an extremely dangerous character and belongs to a subspecies of those allergic reactions that are a state of shock for the human body. Consider how dangerous this syndrome is and how it can be treated.

Characteristics of the disease

This syndrome was first mentioned in 1922. He received the name from the author, who described the main signs of the disease. It can appear at any age, but is more common in people over 20 years of age.

In general, this is a disease of the skin and mucous membranes of the human body caused by allergies. It represents the form when the cells of the epidermis begin to die, as a result - separating from the dermis.

Johnson's syndrome is a malignant exudative erythema that can lead to death. The condition that is caused by the syndrome threatens not only health, but also life. It is dangerous because all the symptoms appear in a matter of hours. We can say that this is a toxic form of the disease.

This syndrome does not proceed like a normal allergic reaction. Bubbles form on the mucous membranes, which literally stick around the throat, genitals, and skin. A person can suffocate because of this, refuse to eat, because. it is very painful, the eyes can stick together, turn sour, and then the bubbles are filled with pus. And I must say that such a state is very dangerous for a person.

The patient with Stevens Johnson's Symptom is in a state of fever, the disease itself progresses at lightning speed - fever, sore throat. These are all just initial symptoms. This is very similar to a cold or SARS, so many simply do not pay attention and do not suspect that it is time to treat the patient.

Often, skin lesions do not occur on one part of the body, and later all the rashes merge together. Progressing, the disease provokes detachment of the skin.

However, doctors state that this pathological condition is very rare and only 5 people out of a million people are prone to pathology. To this day, science is studying the mechanism of development, prevention and treatment of the Syndrome. This is important because people with this condition require emergency medical attention and special care.

Causes of the syndrome

To date, four main causes are known that provoke the development of SJS.

One of them is medication. Most often these are drugs from the category of antibiotics.

  • sulfonamides;
  • cephalosporins;
  • antiepileptic drugs;
  • individual antiviral and non-steroidal anti-inflammatory drugs;
  • antibacterial medicines.

The next cause of SJS is an infection that enters the human body. Among them:

  • bacterial - tuberculosis, gonorrhea, salmonellosis;
  • viral - herpes simplex, hepatitis, influenza, AIDS;
  • fungal - histoplasmosis.

A separate factor provoking SJS is cancer. This syndrome can become a complication of a malignant tumor.

Very rarely, this disease can appear on the background of a food allergy, if substances that can lead to intoxication systematically enter the body.

Even less often, the syndrome develops as a result of vaccination, when the body reacts with increased sensitivity to the components of the vaccine.

However, to this day, medicine does not know why the disease can develop without provoking causes. T-lymphocytes are able to protect the body from foreign organisms, but in the condition that causes the syndrome, these T-lymphocytes are activated against their own body and destroy the skin.

The state of the body caused by the syndrome can be called, rather, an anomaly of the human body. The reactions that it launches develop very rapidly and often for no clear reason.

However, doctors focus on the fact that you should not refuse to take medications provoking the syndrome. Usually, all these drugs are prescribed as a therapy for serious diseases, in which, without treatment, a lethal outcome is possible much faster.

The main thing is that not everyone has an allergy, therefore, the attending physician should make sure of the appropriateness of the appointment, taking into account the patient's medical history.

Symptoms of the syndrome: how to distinguish from other diseases

How quickly the disease will develop will depend on the state of the human immune system. All symptoms may appear in a day, or in a few weeks.

It all starts with an incomprehensible itch and small red spots. The first sign of the development of the syndrome is the appearance of vesicles or bullae on the skin. If you touch them or accidentally touch them, they will simply fall off, leaving purulent wounds behind them.

Then the body temperature rises sharply - up to 40 degrees Celsius, headache, aches, fever, indigestion, redness and sore throat will begin. It should be noted that all this happens in a short period of time. Therefore, it is worth immediately calling an ambulance or urgently taking the patient to the hospital. Delay can cost a person's life.

After the lightning-fast appearance of the above symptoms, small blisters become large. They are covered with a light gray film and a crust of gore. Pathology often develops in the mouth. The patient's lips stick together, so he refuses food and cannot utter a word.

In appearance, this whole picture resembles severe burns of the skin, and the symptoms are similar to a 2nd degree burn. Only the blisters peel off in whole plates and in their place remains wet skin, similar to ichor.

Initially, only a few parts of the body are affected - the face and limbs. Then the disease progresses, and all erosions merge. At the same time, the palms, feet and head remain intact. This fact for doctors becomes the main one in recognizing SJS.

The patient with slight pressure on the skin, from the very first days of the onset of symptoms, will experience severe pain.

An infectious disease can also join the syndrome, which will only aggravate the course of the disease. Another factor in the identification of DDS is eye damage. Due to pus, the eyelids can grow together, conjunctivitis of a serious form will appear. As a result, the patient may lose his sight.

The genitals will not remain intact. As a rule, the development of secondary diseases begins - urethritis, vaginitis, vulvitis. After some time, the affected areas of the skin overgrow, but scars remain, and narrowing of the urethra occurs.

All blisters on the skin will be bright purple in color with an admixture of pus and blood. When they spontaneously open, wounds remain in their place, which then become covered with a rough crust.

The following photographs are examples of what Stevens Johnson Syndrome looks like:

Diagnosis of the disease

In order to correctly diagnose and not confuse the syndrome with another disease, it is necessary to take tests to confirm SJS. This is, first of all:

  • blood chemistry;
  • skin biopsy;
  • Analysis of urine;
  • tank sowing from mucous membranes.

Of course, the specialist will assess the nature of the rashes, and if there are complications, then consultation will be required not only from a dermatologist, but also from a pulmonologist and nephrologist.

Once the diagnosis has been confirmed, treatment should begin immediately. Delay can cost the life of the patient or lead to the development of more serious complications.

Assistance that can be provided at home to the patient before admission to the hospital. Dehydration must be prevented. This is the main thing at the first stage of therapy. If the patient can drink on his own, then you need to give him regularly clean water. If the patient cannot open his mouth, then several liters of saline are injected intravenously.

The main therapy will be aimed at eliminating intoxication of the body and preventing complications. The first step is to stop giving the patient drugs that provoked an allergic reaction. The only exceptions are essential medicines.

After hospitalization, the patient is prescribed:

  1. hypoallergenic diet- food should be slaughtered through a blender or liquid. In a severe case, the body will be replenished intravenously.
  2. Infusion therapy- introduce saline and plasma-substituting solutions (6 liters per day of isotonic solution).
  3. Provide complete room sterility so that no infection can get into the opening of the wound.
  4. Regular cleaning of wounds with disinfectant solutions and mucous membranes. For the eyes, azelastine, with complications - prednisolone. For the oral cavity - hydrogen peroxide.
  5. Antibacterial, pain relievers and antihistamines.

The basis of treatment should be hormonal glucocorticoids. Often, the patient's oral cavity is affected immediately and he cannot open his mouth, so the drugs are administered by injection.

To purify the blood from toxic substances, the patient undergoes plasma filtration or membrane plasmapheresis.

With proper therapy, doctors usually give a positive prognosis. All symptoms should subside within 10 days of starting treatment. After some time, the body temperature will drop to normal, and inflammation from the skin, under the influence of drugs, will subside.

Full recovery will come in a month, no more.

Prevention methods

In general, the usual precautions are the prevention of the disease. These include:

  1. Physicians are prohibited from prescribing for treatment a drug that the patient allergy.
  2. Should not be used drugs from the same group, as well as medicines to which the patient is allergic.
  3. Do not use at the same time a lot of medicines.
  4. Always better to follow instructions on the use of drugs.

Also, those who have weak immunity and those who have already suffered SJS at least once should always remember to take care of themselves and pay attention to alarm bells. Predicting the development of the syndrome is difficult.

If you follow preventive measures, then it will be possible to avoid complications and the rapid development of the disease.

Of course, you should always monitor your health - regularly harden so that the body can resist diseases, use antimicrobial and immunostimulating drugs.

Don't forget about nutrition. It must be balanced and complete. A person must receive all the necessary vitamins and minerals so that they are not in short supply.

The main guarantee of effective treatment is urgent therapy. Everyone who is in the risk category should remember this and without delay in case of suspicious symptoms, seek medical help.

The main thing is not to panic and take the first important steps at the initial stage of the disease. Erythema multiforme exudative is very rare, and exacerbation usually occurs during the off-season - in autumn or spring. The disease develops in both men and women aged 20 to 40 years. However, there are cases when the symptom was found in children under 3 years of age.

If you know about the risks, then you can protect yourself from many complications of the syndrome, which can bring many health problems.

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