Symptoms and treatment of stevens-johnson syndrome. Stevens Johnson syndrome: is the disease as terrible as it looks Stevens Johnson's symptom

Inflammation of the mucous membranes, as well as bullous skin lesions, is called Steven-Jones syndrome. At the same time, the human condition worsens significantly, severe symptoms appear. If the course of the inflammatory process is not stopped in time, then there is a risk of damage to the mucous membranes of the mouth, eyes and organs of the genitourinary system. Ulcers of various diameters form on the patient's skin, deeply affecting the skin.

Steven-Jones syndrome was discovered in 1922 by doctors Stephen and Jones. The disease is not limited to a certain age category, but most often the age of patients is in the range of 20-30 years. Rarely enough, but still not excluded, the occurrence of the syndrome in children under the age of three. If we turn to statistics, then the prevailing majority of patients are males.

Causes of Steven-Johnson Syndrome

The cause of the syndrome is an allergy, which can manifest itself as a result of taking medications, penetration of an infection into the body, tumor formations of organs and tissues. There are also a number of still unidentified causes that may result in Steven-Jones syndrome.

Until the age of 3, Steven-Jones syndrome appears after suffering acute viral infectious diseases: herpes, measles, influenza, chicken pox. It has been established that a bacterial infection in the form of tuberculosis, or mycoplasmosis, can also be a provoking factor for the onset of the disease. It is not excluded the formation of the disease and as a result of a fungal infection - trichophytosis and histoplasmosis.

If you have symptoms of Steven-Jones syndrome, then you need to remember what medications you have taken recently. If these are various types of antibiotics, stimulants of the nervous system and some sulfonamides, then it is possible that they provoked an allergic reaction. In this case, be sure to pay attention to the side effects that are indicated in the instructions for the drug. In the absence of reasons for diagnosing Steven-Jones syndrome, this disease is classified as an idiopathic form of the disease.

Symptoms of Steven-Jones Syndrome

It is worth noting that Steven-Jones syndrome has a rather acute clinical picture. His symptoms develop quite quickly and rapidly. The onset of the disease can remind the patient of the onset of an inflammatory process similar to SARS. But, after a few days, a person realizes that this is not an acute respiratory disease, but something else. After all, an allergic reaction, manifested in the form of rashes on the skin of the face and body, indicates the presence of some other disease.

Inflammation on the skin is symmetrical. Visually, these are small bubbles on the skin with a diameter of 5 cm of a pinkish color. Each such wound is filled with hemorrhagic fluid. With a slight touch to this wound, the bubble opens, the liquid flows out, and erosion forms in its place. The wound begins to itch and hurt badly. Within a few weeks, the patient begins to suffer from bouts of severe coughing, suffocation. Throughout the body notes weakness against the background of general fatigue and fever.

Clinical picture of the disease consists in:

• Strong headache;
• Weakness;
• Chills, fever;
• Pain in muscles and joints;
• Violation of the heart rhythm;
• Pain and;
• Disorder of the digestive tract;
• Cough;
• Bubbles with a clear liquid near the oral mucosa;
• Inflamed border of the lips;
• Difficulties in eating - it becomes painful for a person not only to swallow, but also to drink water.

Numerous ulcers appear on the skin of the face, feet, legs, as well as on the organs of the reproductive system.

Often, Steven-Jones syndrome affects the mucous membrane of the eyes. It may seem to the patient that the eye lens is affected by conjunctivitis, but this is not so. The eyes will not be filled with protein formation, but with pus.

The reproductive system of both men and women is affected by Steven-Jones syndrome in the form of diseases such as urethritis, vulvitis and varying degrees.

Ulcers that occur on the body have a fairly long period of renewal and healing of the affected tissues. This period can take up to several months.

Complications of Steven-Jones Syndrome:

• Uterine bleeding in women;
• Complete or partial loss of vision;
• kidney failure;
• Lethal outcome in 20% of cases.

Diagnosis of Steven-Jones Syndrome

As soon as a person has the clinical picture described above, urgent medical care is necessary. Physicians should immediately carry out infusion therapy.

If the symptoms are minor, then the patient is sent for a full medical examination. The patient needs to take a biochemical blood test, take a biopsy of ulcerative inflammation and make a coagulogram. To confirm the diagnosis, a person is sent for an ultrasound of the pelvic organs.

Treatment of Steven-Jones Syndrome

There are three directions in the treatment of Steven-Jones syndrome. This is infusion and glucocorticoid therapy, as well as hemocorrection.

It is also necessary to provide medical treatment with antibacterial drugs. If the patient's condition is critical, then doctors act on the body with hormonal therapy through intravenous injection.

It is imperative to remove toxins from the patient's body that can poison the body. For this purpose, the patient is infused with blood plasma into the body. For superficial treatment of ulcers, ointments are prescribed, which contain adrenal hormones.

Treatment of the mucous membrane of the eyes begins after a professional consultation with an ophthalmologist. As a rule, it is carried out medically using solutions of albucid and hydrocortisone.

If treatment and hormonal therapy are carried out in a timely manner, then the chances of the patient's recovery increase dramatically.

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 taking 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 taking 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.

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: antibiotics of the penicillin series, 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 mouth 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

Stevens-Johnson Syndrome

All diseases affect everyday life, cause physical discomfort, oppress psychologically.

Respiratory diseases reduce performance, if the pathology also affects the skin and mucous membranes, an aesthetic component is added.

But some diseases that have similar symptoms are life-threatening and require immediate medical attention. One of them is Stevens-Johnson syndrome.

What is Stevens-Johnson Syndrome.

Stevens-Johnson syndrome is a progressive malignant exudative erythema (reddening of the skin) that can be fatal. This life-threatening condition is a lightning-fast allergic reaction.

A characteristic manifestation is the formation of vesicles on the mucous membranes and skin due to the death of epidermal cells, their separation from the dermis, and the formation as a result.

It occurs mainly in people aged 20-40 years, more often males. In children under 3 years of age, diseases are rarely recorded, but isolated cases have been recorded even in newborns.

Stevens-Johnson syndrome is characterized by sharp turning with rapid development of life-threatening complications. This necessitates the prompt provision of medical care.

Causes

Causes that may lead to this dangerous condition include:

• Weakening of the body due to infectious diseases. This leads to a sharp decrease in the effectiveness of the immune system. The following diseases can provoke the development of pathology:
  • bacterial infections (, etc.);
  • viral infections (herpes, hepatitis, human immunodeficiency virus, etc.);
  • fungal diseases ( , histoplasmosis, etc.).
• The intake of certain drugs in the body:
  • drugs for the treatment of gout (allopurinol);
  • non-steroidal anti-inflammatory drugs (ibuprofen);
  • antibacterial drugs (penicillin, sulfonamides: biseptol, sinersul, etc.);
  • anticonvulsants and neuroleptics;
  • radiation therapy.
• Oncological diseases
• In rare cases, the syndrome can develop as a result of exposure to food allergens, ingestion of chemicals, and as a complication of vaccination.

A single cause, or a combination of them, can provoke the development of this syndrome, and in adults this condition is more often caused by taking medications, and in children - by infectious diseases.

It should be noted that the presence of a drug on the list of medications that can cause Stevens-Johnson syndrome is not a reason to refuse a doctor's appointment.

These drugs are used to treat serious diseases, without proper medical care, the risk of complications of these diseases is higher than the risk of developing Stevens-Johnson syndrome, not everyone has an allergic reaction to these drugs.

Therefore, the appropriateness of prescribing such drugs should be assessed by the doctor, taking into account all factors and the patient's medical history.

Risk Factors for Stevens-Johnson Syndrome

Risk factors do not cause Stevens-Johnson syndrome, but can significantly increase the risk of developing it, among them:

• weakening of the immune system;
• medical history - previously suffered Steven Jones syndrome increases the risk of this condition in the future;
• heredity - if one of the family members suffered from this syndrome, this also increases the risk of a similar reaction

Symptoms

Stevens-Johnson syndrome is characterized by an acute onset and lightning-fast development of symptoms.

At the initial stage disease is marked:

• weakness;
• temperature up to 40 degrees;
• cardiopalmus;
• joint pain;
• muscle pain;
• sore throat, perspiration, cough;
• diarrhea, vomiting.

A few hours later the following symptoms are added:

• The appearance of a rash on certain areas of the skin, itching (see photo above). An important sign for diagnosis is that rashes do not appear on the scalp, palms and soles, the predominant localization is the limbs, chest, and back.
• The rash develops into large purple blisters (up to 3-5 cm), after opening which bright red erosions form.
• Blisters appear on the mucous membranes, their opening reveals defects with a white or yellow film, covered with dried blood.
• The defeat of the red border of the lips - patients can not eat and drink. It's hard for them to talk.
• Eye damage is initially allergic in nature, but if an infection joins, purulent inflammation may form. A distinctive feature of Stevens-Johnson syndrome is the formation of small ulcers on the mucous membrane of the eyes, which can develop (inflammation of the cornea), iridocyclitis (inflammation of the iris), (inflammation of the eyelid).
• Damage to the organs of the genitourinary system occurs in half of the cases, due to scarring after inflammation, urethral stricture can develop - narrowing up to obstruction.

New ulcers form within 2-3 weeks, it takes more than 1 month to restore the damaged skin.

Complications

Stevens-Johnson syndrome is dangerous not only for its threatening manifestations, this disease can cause dangerous complications:

• from the respiratory system:,;
• from the genitourinary system: , , , acute liver failure;
• from the digestive system:;
• from the side of vision:, iridocyclitis,;
• cosmetic defects - after healing of erosion, scars form.

Complications in 10% of cases cause death.

Diagnostics

Diagnosis at an early stage of the disease is very important, it allows you to quickly improve the patient's condition.

This dangerous syndrome can be determined on the basis of anamnesis (patient survey), characteristic symptoms (examination), tests, skin biopsy and some instrumental studies (CT, ultrasound, fluoroscopy).

• Questioning the patient helps to identify the causative factor: taking medications, an infectious disease.
• A thorough examination of the skin and mucous membranes reveals characteristic manifestations: a specific rash, its characteristic localization.
• The result of a general blood test indicates nonspecific signs of inflammation: an increase in the level of leukocytes, a decrease in eosinophils, an increased.
• Biochemical analysis shows a decrease in the content of proteins in the blood due to the fraction of albumins, urea, aminotransferases.
• An immunological blood test is very important in the diagnosis. According to its results, an increase in the level of T-lymphocytes and specific antibodies is noted.
• shows a decrease in blood clotting.
• A histological examination of the skin - a biopsy - determines the necrosis of epidermal cells, the subepidermal arrangement of blisters.
• Computed tomography, ultrasound examination of the organs of the genitourinary system and fluoroscopy of the lungs show the presence of a process in the relevant organs.

In complicated cases, consultation may be required. nephrologist, pulmonologist and other specialists.

Treatment

Establishing a diagnosis of progressive malignant exudative erythema is a signal for the immediate start of treatment. Any delay increases the likelihood of severe complications.

Medical care that needs to be provided to the patient can be divided into pre-hospital care and hospital care.

Assistance at the prehospital stage:

• Replenishment of fluid deficiency is the main event at this stage. 1-2 liters of saline solutions are administered intravenously to the patient, if he can drink, it is also necessary to carry out oral rehydration.
• In the early stages of the disease, glucocorticosteroids (prednisolone 60-150 mg) are injected intravenously. The expediency of the subsequent administration of these drugs is questionable - there is a possibility of developing septic complications, so the possibility of further use of prednisolone is determined in each case individually.
• Preparedness for emergency tracheotomy and mechanical ventilation.

Help in a hospital is aimed at continuing fluid replenishment, preventing the development of complications, eliminating all toxic effects, including the abolition of all medications, except for the necessary ones.

Appointed:

• Infusion therapy (isotonic solution, up to 6 liters per day).
• Intravenous glucocorticosteroids as needed.
• Sterile conditions to exclude bacterial infection.
• Treatment of the skin: drying and disinfection, as the wounds heal, disinfectant solutions are replaced with ointments (anti-inflammatory: elocom, locoid, celestoderm).
• Mucosal treatment:
  • eye (azelastine), in severe cases - prednisolone;
  • oral cavity (solutions for disinfection, hydrogen peroxide);
  • genitourinary system (glucocorticosteroid ointments, solutions for disinfection).
• Antihistamines for severe itching.
• Hypoallergenic diet (ban on citrus fruits, fish, nuts, poultry meat, chocolate, alcohol, smoked products, coffee, spices, honey), drink plenty of water.

Forecast

In the case of early diagnosis of Stevens-Johnson syndrome and initiation of treatment, the prognosis is favorable, but often the resulting complications make therapy difficult.

The syndrome refers to a very severe, dangerous pathology, it is necessary to start treatment in a timely manner, traditional medicine methods are powerless in this case, only urgent medical care can help the patient.

In the absence of specialized assistance, the percentage of deaths is high.

Prevention

To reduce the likelihood of developing this dangerous condition, it is necessary to follow a number of measures: giving up bad habits, proper nutrition based on healthy food intake, timely treatment of infectious diseases and, most importantly, taking medications only as prescribed by a doctor.

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Interesting

Bullous erythema multiforme (L51.1), Toxic epidermal necrolysis [Lyella] (L51.2)

Dermatovenereology

general information

Short description

RUSSIAN SOCIETY OF DERMATOVENEROLOGISTS AND COSMETOLOGISTS

Moscow - 2015

Code according to the International Classification of Diseases ICD-10
Stevens-Johnson Syndrome - L51.1
Toxic epidermal necrolysis - L51.2

DEFINITION
Stevens-Johnson syndrome / toxic epidermal necrolysis - epidermolytic drug reactions (ELR) - acute severe allergic reactions, characterized by extensive lesions of the skin and mucous membranes, induced by the use of drugs.

Classification

Depending on the area of ​​the affected skin, the following forms of ELR are distinguished:
- Stevens-Johnson syndrome (SSD) - less than 10% of the body surface;
- toxic epidermal necrolysis (TEN, Lyell's syndrome) - more than 30% of the body surface;
- an intermediate form of SJS / TEN (damage to 10-30% of the skin).

Etiology and pathogenesis

Most often, Stevens-Johnson syndrome develops when taking medications, but in some cases the cause of the disease cannot be found out. Among the drugs that are more likely to develop Stevens-Johnson syndrome, there are: sulfonamides, allopurinol, phenytonin, carbamazepine, phenibutazole, piroxicam, chlormazanone, penicillins. Less often, the development of the syndrome is facilitated by the use of cephalosporins, fluoroquinolones, vancomycin, rifampicin, ethambutol, tenoxicam, thiaprofenic acid, diclofenac, sulindac, ibuprofen, ketoprofen, naproxen, thiabendazole.
The incidence of ELR is estimated at 1-6 cases per million people. ELR can occur at any age, the risk of developing diseases increases in people over the age of 40, in HIV-positive people (1000 times), patients with systemic lupus erythematosus and oncological diseases. The older the patient, the more serious the concomitant disease, and the more extensive the skin lesion, the worse the prognosis of the disease. Mortality from ELR is 5-12%.
Diseases are characterized by a latent period between taking the drug and the development of the clinical picture (from 2 to 8 weeks), which is necessary for the formation of an immune response. The pathogenesis of ELR is associated with massive death of basal keratinocytes in the skin and mucosal epithelium caused by Fas-induced and perforin/granzyme-mediated cell apoptosis. Programmed cell death occurs as a result of immune-mediated inflammation, in which cytotoxic T cells play an important role.

Clinical picture

Symptoms, course

In Stevens-Johnson syndrome, there is damage to the mucous membranes of at least two organs, the area of ​​the lesion reaches no more than 10% of the entire skin.

Stevens-Johnson syndrome develops acutely, damage to the skin and mucous membranes is accompanied by severe general disorders: high body temperature (38 ... 40 ° C), headache, coma, dyspeptic symptoms, etc. Rashes are localized mainly on the skin of the face and trunk. The clinical picture is characterized by the appearance of multiple polymorphic rashes in the form of purple-red spots with a bluish tinge, papules, vesicles, and target-like foci. Very quickly (within a few hours), bubbles are formed on these places up to the size of an adult's palm and more; merging, they can reach gigantic sizes. Limes of blisters are relatively easily destroyed (positive symptom of Nikolsky), forming extensive bright red eroded weeping surfaces, bordered by fragments of blisters tires ("epidermal collar").
Sometimes rounded dark red spots with a hemorrhagic component appear on the skin of the palms and feet.

The most severe lesion is observed on the mucous membranes of the oral cavity, nose, genital organs, the skin of the red border of the lips and in the perianal region, where blisters appear, which quickly open, exposing extensive, sharply painful erosions covered with a grayish fibrinous coating. On the red border of the lips, thick brown-brown hemorrhagic crusts often form. If the eyes are affected, blepharoconjunctivitis is observed, there is a risk of developing corneal ulcers and uveitis. Patients refuse to eat, complain of pain, burning, hypersensitivity when swallowing, paresthesia, photophobia, painful urination.

Diagnostics

The diagnosis of Stevens-Johnson syndrome is based on the results of the anamnesis of the disease and the characteristic clinical picture.

When conducting a clinical blood test, anemia, lymphopenia, eosinophilia (rarely) are detected; neutropenia is an unfavorable prognostic sign.

Carry out if necessary histological examination skin biopsy. Histological examination reveals necrosis of all layers of the epidermis, the formation of a gap above the basement membrane, detachment of the epidermis, and the inflammatory infiltration in the dermis is insignificant or absent.

Differential Diagnosis

Stevens-Johnson syndrome should be differentiated from pemphigus vulgaris, staphylococcal scalded skin syndrome, toxic epidermal necrolysis (Lyell's syndrome), which is characterized by epidermal detachment of more than 30% of the body surface; graft-versus-host disease, erythema multiforme exudative, scarlet fever, thermal burn, phototoxic reaction, exfoliative erythroderma, fixed toxidermia.

Treatment

Treatment Goals

Improving the general condition of the patient;
- regression of rashes;
- prevention of development of systemic complications and relapses of the disease.

General notes on therapy
Treatment of Stevens-Johnson syndrome is carried out by a dermatovenereologist, therapy for more severe forms of ELR is carried out by other specialists, a dermatovenereologist is involved as a consultant.
If an epidermolytic drug reaction is detected, the doctor, regardless of his specialization, is obliged to provide the patient with emergency medical care and ensure his transportation to the burn center (department) or to the intensive care unit.
Immediate discontinuation of the drug that provoked the development of ELR increases survival with a short half-life. In doubtful cases, all non-essential drugs should be discontinued, and especially those started within the last 8 weeks.
It is necessary to take into account the unfavorable prognostic factors for the course of ELR:
1. Age > 40 years - 1 point.
2. Heart rate > 120 per minute - 1 point.
3. Defeat > 10% of the skin surface - 1 point.
4. Malignant neoplasms (including history) - 1 point.
5. In a biochemical blood test:
- glucose level > 14 mmol/l - 1 point;
- urea level > 10 mmol/l - 1 point;
- bicarbonates< 20 ммоль/л - 1 балл.
Probability of death: 0-1 points (3%), 2 points (12%), 3 points (36%), 4 points (58%), >5 points (90%).

Indications for hospitalization
Established diagnosis of Stevens-Johnson syndrome / toxic epidermal necrolysis.

Treatment regimens

Systemic therapy
1. Glucocorticosteroid preparations of systemic action:
- prednisolone (B) 90-150 mg per day intramuscularly or intravenously
or
- dexamethasone (B) 12-20 mg per day intramuscularly or intravenously.

2. Infusion therapy(alternation of different schemes is acceptable):
- potassium chloride + sodium chloride + magnesium chloride (C) 400.0 ml intravenously drip, for a course of 5-10 infusions
or
- sodium chloride 0.9% (C) 400 ml intravenously drip for a course of 5-10 infusions
or
- calcium gluconate 10% (C) 10 ml 1 time per day intramuscularly for 8-10 days;
- sodium thiosulfate 30% (C) 10 ml 1 time per day intravenously for a course of 8-10 infusions.
It is also justified to carry out procedures of hemosorption, plasmapheresis (C).

3. In the event of infectious complications, appoint antibacterial drugs taking into account the isolated pathogen, its sensitivity to antibacterial drugs and the severity of clinical manifestations.

External Therapy
consists in careful care and treatment of the skin by cleansing, removing necrotic tissue. Extensive and aggressive excision of the necrotic epidermis should not be undertaken because superficial necrosis is not a barrier to re-epithelialization and may accelerate stem cell proliferation via inflammatory cytokines.
For external therapy, solutions of antiseptic preparations are used (D): hydrogen peroxide solution 1%, chlorhexidine solution 0.06%, potassium permanganate solution (D).
For the treatment of erosion, wound dressings, aniline dyes (D): methylene blue, fucorcin, brilliant green are used.
In case of damage to the eyes, an ophthalmologist's consultation is required. It is not recommended to use eye drops with antibacterial drugs due to the frequent development of the "dry eye" syndrome. It is advisable to use eye drops with glucocorticosteroid drugs (dexamethasone), artificial tears. Requires mechanical destruction of early synechiae in case of their formation.
If the oral mucosa is affected, rinse several times a day with antiseptic (chlorhexidine, miramistin) or antifungal (clotrimazole) solutions.

Special situations
Treatment of children
Requires intensive interdisciplinary interaction of pediatricians, dermatologists, ophthalmologists, surgeons:
- control of fluid balance, electrolytes, temperature and blood pressure;
- aseptic opening of still elastic bubbles (the tire is left in place);
- microbiological monitoring of lesions on the skin and mucous membranes;
- eye and oral care;
- antiseptic measures, aniline dyes without alcohol are used to treat erosions in children: methylene blue, brilliant green;
- non-adhesive wound dressings;
- placing the patient on a special mattress;
- adequate analgesic therapy;
- careful therapeutic exercises to prevent contractures.

Requirements for treatment outcomes
- clinical recovery;
- prevention of recurrence.

PREVENTION
Prevention of recurrence of Stevens-Johnson syndrome consists in the exclusion of drugs that caused this disease. It is recommended to wear an identification bracelet indicating the drugs that caused Stevens-Johnson syndrome.

Information

Sources and literature

1. Clinical recommendations of the Russian Society of Dermatovenerologists and Cosmetologists
   1. 1. Fine J.D: Management of acquired bullous skin diseases. N Engl J Med 1995; 333: 1475–1484 2. Kardaun S.H., Jonkman M.F. Dexamethasone pulse therapy for Stevens-Johnson syndrome/toxic epidermal necrolysis. Acta Derm Venereol 2007; 87:144–148. 3. Kamanbroo D., Schmitz-Landgraf W., Czarnetski B.M. Plasmapheresis in severe drug-induced toxic epidermal necrolysis. Arch Dermatol 1985; 121: 1548–1549.

Information

The personal composition of the working group for the preparation of federal clinical guidelines for the profile "Dermatovenereology", section "Steven-Johnson Syndrome":
1. Zaslavsky Denis Vladimirovich - Professor of the Department of Dermatovenereology, St. Petersburg State Pediatric University of the Ministry of Health of Russia, Professor, Doctor of Medical Sciences, St. Petersburg.
2. Gorlanov Igor Aleksandrovich Head of the Department of Dermatovenereology, St. Petersburg State Pediatric University of the Ministry of Health of Russia, Professor, Doctor of Medical Sciences, St. Petersburg.
3. Aleksey Viktorovich Samtsov - Head of the Department of Skin and Venereal Diseases, Military Medical Academy named after S.M. Kirov”, doctor of medical sciences, professor, St. Petersburg.
4. Khairutdinov Vladislav Rinatovich - Assistant of the Department of Skin and Venereal Diseases, Military Medical Academy named after S.M. Kirov, Doctor of Medical Sciences, St. Petersburg.

METHODOLOGY