Malignant exudative erythema stevens johnson syndrome. Stevens Johnson Syndrome and Lyell's Syndrome. Prevention of the development of Lyell's syndrome

Stevens-Johnson syndrome is a chronic disease characterized by hyperemia and rashes on the skin and mucous membranes.

This pathological condition has several clinical manifestations. That is why in different sources it can be called differently.

In order to learn how to cope with the problem, you need to know what can provoke its occurrence.

According to the statistics of the World Health Organization, this painful condition most often occurs in childhood from 3 to 14 years old, as a complication of the underlying disease. Until the age of 3, only isolated cases are recorded, but the pathology in an infant is especially difficult.

The causes of the appearance of Stevens-Johnson syndrome in children include.

1. Viral infection (herpes, hepatitis, measles, chicken pox, mumps, influenza).
2. Bacterial infection (tuberculosis, piroplasmosis, salmonellosis).
3. Infection caused by a fungus (candidiasis, coccidiosis, trichophytosis).
4. Drug allergy caused by taking antibiotics, vitamins, hormonal drugs.

In more mature age, the main cause is severe immunodeficiency, which can be caused by the following diseases.

1. Oncological diseases of the skin, lymph nodes.
2. HIV infections.
3. Severe form of tuberculosis.
4. Severe forms of allergies (Quincke's edema, dermatitis, eczema).

It is very difficult to establish a diagnosis in a timely manner. This is due to the fact that in the initial stage the pathological process is hidden under the guise of many diseases.

These include:

• SARS;
• allergic dermatitis;
• erythema of various forms and locations.

However, after 5-7 weeks, true symptoms appear, which make it possible to unambiguously say that this is this pathology.

There are certain stages in the development of a painful condition in Stevens-Johnson syndrome.
The clinical picture at the beginning of the development of Stevens-Johnson syndrome consists of flu-like symptoms, namely:

• severe pain all over the body;
• general weakness;
• ailments;
• sore throat;
• high fever with periods of fever.

In severe cases, the patient complains of cough, catarrhal discharge from the nose, diarrhea. It starts suddenly, without any prerequisites.

After 2 - 3 days, the first elements of rashes appear on the skin, after which the temperature drops as sharply as it appears:

• initially these are single bubbles, up to 2 centimeters in diameter;
• then they rapidly diverge over the entire surface of the skin and mucous membranes of the internal organs.

On the 4th - 6th day after the onset of the disease, the clinical picture depends on the location of the blistering rashes, their number, and size. You can most clearly see the manifestations of Stevens-Johnson syndrome in the photo.

Damage to the mucous membrane of the eyes is characteristic of the disease:

• there is a sensation of a foreign body in the eye;
• then the conjunctiva becomes covered with small blistering rashes;
• there is a sharp pain when blinking, purulent discharge from the eyes appears.

Untimely treatment inevitably leads to damage to the outer shell of the eye, the cornea. The formation of blisters on the mucous membrane of the mouth or nasopharynx leads to the fact that the patient cannot eat. Rashes are very similar to the manifestation of stomatitis. However, with Stevens-Johnson disease, not only the oral cavity is affected, but also the lips.

If the process extends to more than two internal organs, then the disease is called exudative erythema multiforme.

It is especially difficult to diagnose damage to the mucous membrane of the urethra, vagina, nose. This is due to the fact that it is practically impossible to see the rash visually.

This pathological condition can be treated only by general methods, without the use of ointments and creams.

After opening the bubbles, they begin to dry. Then comes the recovery period. The main thing at this stage is to avoid scratching, as well as the addition of a secondary infection.

Lyell's syndrome has a similar clinical picture. Lyell and Stevens-Johnson syndrome can be distinguished using differential diagnosis. The main difference is that with Lyell's syndrome, the skin looks like after a burn with hot water.

Timely treatment plays a special role in the successful outcome of the disease. In Russia, about 10% of cases of this pathology are fatal, and another 30-40% of cases of the transition of the disease to malignant exudative erythema.

Initially, it is necessary to cure the underlying problem that provoked the appearance of a painful skin condition. Only then can you fight Stevens-Johnson disease.

Drugs used for treatment, the principle of their action.

An acute bullous lesion of the mucous membranes and skin of an allergic nature is called Stevens-Johnson syndrome. The disease affects the genitourinary organs, eyes, oral mucosa. Data on the syndrome were first published in 1922, over time it was named after the authors who described it. Dermatology classifies Stevens-Johnson syndrome as bullous dermatitis.

In medicine, Stevens-Johnson syndrome is often called malignant exudative erythema. The causes of this disease may be the following:

• Medicines such as antibiotics, vitamins, non-steroidal anti-inflammatory drugs, vitamins, topical anesthetics, and other drugs.
• Pathogenic bacteria and other infectious agents.
• Some cancers, such as lymphomas or carcinomas.
• Unknown reasons.

Despite the presence of some ideas about the etiology of this form of an allergic reaction, its pathogenesis is not fully understood. Medical staff only know that when this syndrome appears, allergic activity increases sharply, which becomes aggressive towards its own structures of the body, in this case, the skin.

Pathology develops rapidly, but the first symptoms are quite diverse. They include general malaise, a sharp increase in body temperature up to 40 ° C, headaches, heart palpitations, pain in the joints and muscles. But there may also be sore throat, coughing, vomiting or diarrhea.

When several hours to a day pass from the onset of the disease, changes appear on the mucous membranes of the oral cavity and organs of the genitourinary system. Large blisters form in the mouth, which after a while open up and instead of them there are large wounds with a film of a white-gray or yellow tint or a crust of gore. All this greatly prevents the patient from speaking and does not allow him to drink and eat normally.

As for the organs of the genitourinary system, their defeat with its symptoms resembles urethritis, balanoposthitis, vulvitis or vaginitis. And if erosions and ulcers on the urethral mucosa begin to scar, there is a risk of strictures.

Skin lesions are the appearance of a large number of round, blister-like rash elements of a bright purple hue, located mainly on the trunk and in the perineum. Their sizes can reach 5 cm, and their feature is that in the center of such an element there are serous or bloody blisters. When the blisters open, red wounds remain in their place, which eventually become covered with crusts.

Before the eyes, Stevens-Johnson syndrome appears initially as allergic conjunctivitis, which is often complicated by secondary infection and purulent inflammation. Small erosions and ulcers appear in the conjunctiva and cornea of ​​the eye. Sometimes the iris is also affected, blepharitis, iridocyclitis, or even keratitis develops.

New elements of the rash appear about 2-3 weeks, and the healing of ulcers after them occurs within one and a half months. The patient's condition may worsen due to frequent complications, such as:

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

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 causing Steven Johnson's syndrome: 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.

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. Men are more often affected 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, manifest themselves 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 begins to be diagnosed with anamnesis. To do this, the doctor asks the patient questions, for example: “Have you suffered from allergic diseases before, and which ones? What symptoms were they and their causes?”, “Did the patient try to treat this disease on his own and by what methods: medication or folk remedies?”, As well as other similar questions.

At the beginning of the diagnosis, the doctor examines the skin and mucous membranes throughout the body and notes changes, characterizes the rash that has appeared. Views the placement, the appearance of minor skin neoplasms. The doctor looks at whether there is shortness of breath, the state of blood pressure, disorders of the gastrointestinal tract, in the urine excretory system.

During an objective examination, heart rate, blood pressure, temperature are measured, lymph nodes and the abdominal cavity are pulped.

After all this, laboratory tests are carried out. Every day you need to donate blood for a detailed general analysis, it is given until the patient's condition is stable. A coagulogram is carried out, a general urine test is also taken daily, samples of the affected skin and mucous membranes are taken. A biochemical blood test is also carried out.

In general, the patient turns to an allergist or dermatologist, but if certain internal organs are affected, one should contact doctors with a narrow subject.

Video: Symptoms of erythema multiforme exudative

Erythema multiforme exudative requires first pre-hospital care. At the beginning of the development of this disease, a lot of fluid is lost, to stop this condition, peripheral vein catheterization is performed, and fluid transfusion is used for this, saline solutions in a volume of one to two liters.

Corticosteroids are administered in the form of injections: intravenous prednisone. This is a hormonal drug and may not be very effective in this case. It is necessary to carry out artificial ventilation of the lungs.

Treatment is mainly carried out in a hospital.

First of all, corticosteroids are used. They are very strong and stop the allergic reaction from expanding further. In most cases, they are administered in the form of injections, they begin to act faster. Corticosteroids are examples: prednisolone, dexamethasone. In combination with them, electrolyte solutions are prescribed. It is desirable to administer them in the form of droppers, the medicine is administered in this way.

Dubin John syndrome affects a very large area of ​​the skin and as a result of this, a viral complication may appear. To eliminate their manifestations, antibiotic therapy is used. The drugs themselves in the form of antibiotics are selected individually for each patient.

You can not do without local treatment of Steven Johnson's syndrome, it includes proper skin care and careful removal of inanimate skin. This requires antiseptic solutions. They are used in the form of peroxide and potassium permanganate.

Depending on the location of the localization, an individual treatment of the organ is prescribed. If the oral cavity is affected, after each meal it is treated with peroxide or other disinfectant solutions. Urinary excretory organs are treated with solcoseryl ointment or prednisolone, three times a day. Eyes are smeared with eye ointments, eye drops are applied.

There is one of the basic principles of treatment, it is to find out the cause, which gave rise to Steven Johnson's syndrome. You must follow a hypoallergenic diet. Sweets, chocolate, fish products, citrus fruits, chicken meat, coffee, spices and mayonnaise, strawberries, melons, honey, alcohol are excluded from the diet.

Polymorphic exudative erythema is a disease in which the vessels of the skin are affected, as a result of which the skin and mucous membranes change. Symptoms are: spots that look like a target, bubbles appear. Localized on the hands, feet, and mucous membranes. This form is also called erythema multiforme exudative.

Erythema multiforme exudative develops in younger people. The rash lasts for a week. Appears to be almost red.

1. Complications of various organs:
2. from the gastrointestinal tract - colitis, proctitis;
3. eye diseases - severe keratitis, blindness;
4. urine excretory system - renal failure, bleeding from the genitals;
5. skin - scars, scars;

• In no case should you start treatment with small doses of corticosteroids, and you should not use them for a long time after the end of treatment.
• If an infectious disease has not joined, you should not use antibiotics at all.
  The treatment algorithm should not include penicillin antibiotics and vitamin preparations. They are considered strong allergens.
• The treatment of Stevens Johnson syndrome is similar to the standard treatment for allergies, but since this is a more severe disease, you must immediately consult a doctor and he already prescribes individual treatment for each patient.

Video: Erythema multiforme exudative - a rare and very serious disease

Stevens Johnson syndrome: is the disease as terrible as it looks

Among seemingly harmless allergic reactions, 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.

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.

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.

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.

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.

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:

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. First of all, the patient is stopped giving 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.

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.

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 ofmedicines.
4. Always better to followinstructions 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, you can protect yourself from many complications of the syndrome, which can bring many health problems.

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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 the development of 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 form in 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 dermatovenerologist, 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 performed because superficial necrosis does not prevent 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 eye damage, 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, for the treatment of erosion in children, aniline dyes without alcohol are used: 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 Alexandrovich 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

Against the background of viral, bacterial, fungal infections, after the administration of medications, some patients with hypersensitivity of the body develop bullous lesions of the skin and mucous membranes. A severe inflammatory process provokes dangerous complications.

With increased sensitization of the body, it is important to know how Stevens-Johnson syndrome develops, what it is, how to act when identifying signs of a dangerous allergic reaction? Factors that provoke the development of a serious disease, symptoms, methods of treatment and prevention are described in the article.

Reasons for the development of pathology

An acute allergic reaction develops against the background of the following factors:

• taking or administering drugs. A negative response is most often caused by antibiotics (especially penicillins) - more than half of the cases, NSAIDs - up to 25%. The list of potential allergens includes vitamins, sulfonamides, local anesthetics;
• developing cancer;
• penetration of pathogenic microorganisms. The infectious-allergic form of the disease occurs when exposed to viruses, contact with protozoal, fungal infections, bacterial agents;
• idiopathic form of a dangerous reaction. Unexplained etiology of severe disease is from 25 to 50% of cases.

Stevens-Johnson syndrome ICD code - 10 - L51.1 (bullous erythema multimorphic).

First signs and symptoms

Severe allergic disease belongs to the group of bullous dermatitis with a characteristic feature: blisters on the mucous membranes and skin. Other symptoms also appear: negative reactions affect the epidermis, internal organs, lips, eyes, and oral cavity.

The patient's condition, the appearance of the patient resembles the clinical picture after severe burns. The danger of an allergic disease is a high rate of progression of negative manifestations. The dangerous syndrome is an immediate reaction.

How the disease develops:

• an allergic reaction of the immediate type has an acute onset. The early stage is similar to the development of a viral infection: the temperature rises, often up to 39-40 degrees, the head hurts, weakness appears, pain in the joints and muscles, the heartbeat quickens;
• further there is a cough, sore throat, vomiting, diarrhea. After 5-6 hours (no later than a day), the oral mucosa is covered with large blisters. Quite quickly, the blisters open, erosions are formed, covered with gore, yellowish or grayish-white films. The dangerous process spreads further, affecting the lips. For this reason, it is very difficult for patients to drink and eat;
• one of the signs is . A dangerous complication is purulent inflammation with the formation of erosive and ulcerative elements on the cornea and conjunctiva. Eye damage develops;
• purple blisters are visible on the skin. The diameter of the formations is up to 5 cm, in the center of large blisters, bloody or serous zones are visible. After opening, erosion appears, then the affected area is covered with crusts. The main places of rashes are the perineum, various zones of the body;
• in half of patients suffering from a dangerous syndrome, the genital area and urinary system become inflamed. Vaginitis, cystitis, urethritis, balanoposthitis with scarring often leads to damage to the urethra;
• the period of rashes lasts up to three weeks, the affected areas heal for a long time - up to one and a half months. Often, a dangerous disease is accompanied by complications: kidney failure, pneumonia, bleeding from an inflamed bladder, visual impairment, secondary infection, colitis. A massive attack on the body leads to the death of almost 10% of patients.

Effective Treatments

The examination is carried out by a therapist, the patient is necessarily examined by an allergist. It is important to find out which factor provoked a dangerous reaction, which drugs should not be given to the victim during therapy. The doctor finds out whether allergic diseases have occurred before, how the body reacted to stimuli.

The rapid development of the immune response threatens the life of the patient. If the symptoms described in the "Symptoms" section appear, you should not hesitate to call an "ambulance": untimely assistance is dangerous for the patient. If Stevens-Johnson syndrome is suspected, the patient is placed in a hospital, resuscitation is often required.

The main directions of therapy:

• emergency care - to prevent dehydration, as in patients with severe burns. Doctors inject saline and colloidal solutions into a vein, if the patient is able to drink, then the liquid is given orally;
• at the choice of a doctor, glucocorticosteroids are administered (intravenously, by jet) or pulse therapy in the early stages of an allergic reaction. The second option is less toxic to the body, there are less dangerous complications;
• in severe form, the active development of the pathological process may require tracheotomy, artificial ventilation of the lungs. When such reactions occur, the patient is immediately taken to intensive care;
• in the hospital, doctors carry out detoxification, prevent secondary infection, exclude repeated contact with the irritant, especially in the dosage form of allergy;
• mandatory infusion therapy with the introduction of special solutions;
• reduce the load on the body, prevent the action of hazardous types of food helps. It is important to remember that for an allergic person with a severe negative reaction, any amount of inappropriate food can be dangerous;
• it helps to prevent the penetration of harmful bacteria by placing the patient in a ward with sterile conditions, as in a burn unit;
• eliminate the effects of skin reactions disinfectant and saline solutions, corticosteroid, wound healing, emollient creams and ointments. A good effect at the recovery stage is given by hormonal preparations Celestoderm, Elokom, Advantan, Lokoid.

Penicillins, vitamins of group B are categorically contraindicated: these funds increase the risk of allergic reactions, provoke an increase in negative symptoms.

Other therapeutic measures and manipulations are carried out:

• the addition of a bacterial, fungal, viral infection requires the appointment of effective combined ointments. Recommended preparations Belogent, Pimafukort, Triderm;
• antihistamines relieve the sensitivity of histamine receptors, prevent further release of inflammatory mediators. Doctors select antiallergic drugs taking into account the age, condition of the patient, the severity of the reaction. Long-term treatment requires the use of classic antihistamines that quickly remove signs of allergy;
• the inflamed oral cavity after eating must be treated with antiseptics, hydrogen peroxide;
• elimination of negative symptoms in the eyes is carried out with the use of eye drops and gels. Preparations: Oftagel, Azelastine, Prednisolone;
• in case of damage to the organs of the genitourinary system, Solcoseryl ointment, antiseptic solutions are applied to the inflamed areas, in severe rashes - glucocorticosteroids for topical use;
• with severe pain in the muscles and joints, analgesics are needed. The drugs are selected by the doctor depending on the severity of the case.

Go to the address and read information about whether there can be an allergy to frost on the face and how to treat the disease.

Stevens-Johnson syndrome in children

Up to 3 years, doctors rarely register a dangerous allergic disease. The main age category of patients is men aged 20 to 40 years, women get sick less often.

A severe reaction to irritants is dangerous for a growing organism, weak immunity does not allow babies to fight infection. Symptoms of the disease in children are similar to negative manifestations in adults.

At an early age, the main cause of the development of a dangerous disease is the introduction or intake of antibiotics, more often the penicillin series. The reaction is lightning fast, the signs are life threatening.

Immediate medical intervention is required, placing the child in a separate box, ensuring sterility, as in the treatment of patients with burn injuries. Complex therapy is carried out with the use of glucocorticosteroids, saline solutions, wound healing ointments. Mandatory reception, cleansing the body.

On a note:

• after recovery, the patient and relatives should analyze the situation, remember which stimuli led to a negative response of the body. Medication control can help prevent the development of a severe reaction. In the outpatient chart, doctors write down drugs, against the background of which bullous multimorphic erythema arose;
• patients who have suffered an acute allergic reaction are prohibited from taking medications on their own initiative: inappropriate drugs can again cause a dangerous disease - Stevens-Johnson syndrome. The consequences of a repeated attack are often more severe than in the first case of an allergic pathology: it is important to remember what negligence in health can result in.

With increased sensitivity of the body, both mild and acute reactions develop. One of the most dangerous diseases of an allergic nature is Stevens-Johnson syndrome. Erosions, sores, blisters, vomiting, high fever, damage to the mucous membranes and internal organs, deterioration in well-being are far from all the symptoms of a serious illness.

If you suspect the development of a serious illness, the best way out is to call an ambulance to take the patient to the hospital. Home methods, folk remedies, self-medication are not suitable methods of therapy: only qualified doctors can help the patient; in severe cases, resuscitators cannot be dispensed with.

A qualified specialist in the following video will tell you more about Stevens-Johnson syndrome:

Stevens-Johnson syndrome is a bullous lesion of the skin and mucous membranes. It has an allergic nature and is characterized by an acute manifestation.

This disease occurs against the background of a serious condition of a person. It affects the oral mucosa and urinary organs.

Also, this syndrome is called "malignant exudative erythema." As well as, allergic contact, etc. it is a bullous dermatitis and is characterized by a large number of blisters on the skin and mucous membranes.

Most often, this syndrome develops in people in the period from 20 to 40 years. Very rare in children.

Men suffer from this disease more often than women.

The reasons

The causes of the development of Steven Johnson syndrome lie in the immediate allergic reaction of the body. There are four groups of reasons that can cause the onset of such a reaction:

• infections;
• medicines;
• malignant diseases;
• unknown factors.

In children, this syndrome most often develops due to viral diseases (herpes, viral hepatitis, chickenpox, measles, etc.)

Bacterial infections and fungi (tuberculosis, gonorrhea, histoplasmosis, trichophytosis, etc.) can also provoke development.

Adults for the most part suffer from this syndrome due to the use of certain medications or malignant neoplasms in the body.

Of the drugs, most often such a reaction can be caused by antibiotics, regulators of the central nervous system, etc.

The most common cause of cancer is lymphoma or carcinoma.

Symptoms

With the onset of the disease, the symptoms appear very quickly and abruptly. A person notes:

• general malaise;
• the temperature rises to 40c;
• headache;
• arthralgia occurs;
• muscle pain;
• tachycardia.

The patient may have a sore throat, diarrhea or vomiting, cough.

In a few hours, blisters begin to swell in the throat, which, after opening, form large defects. They are covered with white-gray or yellow films and blood crusts.

Lips may also be involved in the process.

Eye damage resembles conjunctivitis, but if an infection gets in, purulent inflammation may develop. It can also lead to blepharitis, keratitis, and iris damage.

Urethritis, vulvitis or vaginitis develops on the genitals.

Numerous elements appear on the skin, which rise above the rest of the skin and have a rounded shape. Outwardly, they look like blisters. They can be up to 5 cm in diameter.

The rash continues to appear for a couple of weeks. Ulcers that remain after opening the blisters heal for a month and a half.

Due to the complications that this syndrome can cause, approximately 10% of patients die.

Diagnostics

Diagnosis of this syndrome includes a large comprehensive study, during which the patient is carefully examined, an immunological blood test is performed, a skin biopsy is taken, and a coagulogram is taken. An x-ray of the lungs, ultrasound of the bladder, kidneys, as well as a biochemical analysis of urine are also performed.

Treatment

Treatment for Stevens Johnson syndrome is complex and intensive. Be sure to prescribe glucocorticoids in large doses. Since these substances can affect the mucous membranes, they are injected. The dose is reduced only after the symptoms subside and the person begins to feel better.

To purify the blood, methods of extracorporeal hemocorrection are used:

• cascade plasma filtration;
• membrane plasmapheresis;
• hemosorption;
• immunosorption.

A person is given a transfusion of plasma and protein solutions.

Be sure to provide the body with plenty of fluids and maintain daily diuresis.

Potassium and calcium preparations are also used.

Secondary infections are treated with antibiotics.

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, other areas of the skin and mucous membranes.

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

What provokes / Causes of 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 a patient very quickly, rapidly, since in fact it is an allergic reaction of an immediate type. Initially, there is a severe fever, pain in the joints and muscles. In the future, after only a few hours or a day, a lesion of the oral mucosa is detected. Here, blisters of rather large sizes appear, skin defects covered with gray-white films, crusts consisting of clotted blood, cracks.

There are also defects in the area of ​​the red border of the lips. Eye damage proceeds according to the type of conjunctivitis (inflammation of the mucous eyes), however, the inflammatory process here is purely allergic in nature. In the future, a bacterial lesion may also join, as a result of which the disease begins to proceed more severely, the patient's condition deteriorates sharply. On the conjunctiva with Stevens-Johnson syndrome, small defects and ulcers may also appear, inflammation of the cornea, posterior parts of the eye (retina, etc.) may join.

Lesions very often can also capture the genitals, which manifests itself in the form of urethritis (inflammation of the urethra), balanitis, vulvovaginitis (inflammation of the female external genital organs). Sometimes mucous membranes are involved in other places. As a result of skin lesions, a large number of redness spots form on it with elevations located on them above the level of the skin in the form of blisters. They have rounded outlines, crimson color. In the center they are cyanotic and seem to sink a little. The diameter of the foci can be from 1 to 3-5 cm. In the central part of many of them, blisters form, which contain a clear aqueous liquid or blood inside.

After opening the blisters, bright red skin defects remain in their place, which then become covered with crusts. Basically, the lesions are located on the body of the patient and in the perineum. The violation of the general condition of the patient is very pronounced, which manifests itself in the form of severe fever, malaise, weakness, fatigue, headache, dizziness. All these manifestations last an average of about 2-3 weeks. In the form of complications during the disease, pneumonia, diarrhea, insufficiency of kidney function, etc. can join. In 10% of all patients, these diseases are very difficult 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, an increase in the erythrocyte sedimentation rate are revealed. These manifestations are very nonspecific and occur in almost all inflammatory diseases. In a biochemical blood test, it is possible to 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 decompose it. The total protein content in the blood also becomes significantly reduced. The most informative and valuable in this case is 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 with 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, from parents and other relatives. The time of the onset of the disease, the effect on the body of various factors that preceded it, especially the intake of medications, are clarified in detail. The external manifestations of the disease are evaluated, for which the patient must be undressed and carefully examined the skin and mucous membranes. 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 for Stevens-Johnson Syndrome:

Mostly, preparations of hormones of the adrenal cortex are used in medium dosages. They are administered to the patient until there is a persistent significant improvement in the condition. Then the dosage of the drug begins to be gradually lowered, and after 3-4 weeks it is completely canceled. In some patients, the condition is so severe that they are not able to take drugs by themselves by mouth. In these cases, hormones are given in liquid form intravenously. Very important are the procedures that are aimed at removing from the body immune complexes circulating in the blood, which are antibodies associated with antigens. For this, special preparations for intravenous administration, methods of blood purification in the form of hemosorption and plasmapheresis are used.

Oral medications are also used to help eliminate toxins 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 in various ways. At the same time, it is ensured that all this volume is removed from the body in a timely manner, since toxins are not washed out during fluid retention and quite serious complications can develop. It is clear that the full implementation of these measures is possible only in the intensive care unit.

Quite an effective measure is the intravenous transfusion of solutions of proteins and human plasma to the patient. Additionally, drugs containing calcium, potassium, antiallergic drugs are prescribed. If the lesions are very large, 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 them. Various antiseptic solutions are used to prevent infection.

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 course of the disease itself, the presence of certain complications.

Which doctors should you contact if you have Stevens-Johnson Syndrome:

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