Stevens-Johnson syndrome (Malignant exudative erythema). Manifestations and therapy of Stevens Johnson syndrome Characteristic signs and symptoms
The name DDS (Lyell and Johnson syndrome) refers to severe inflammation of the skin and mucous membranes - erythema multiforme. The disease was discovered in the USA in 1922. It was described by two pediatricians, Lyell and Johnson, after whom the syndrome got its name. The disease is characterized by an acute course, with damage to the mucous membranes and skin of at least two organs.
Currently, Lyell and Stevens Johnson syndrome occurs worldwide in people predominantly over the age of forty. Recently, the disease has been diagnosed in children and even infants.
Prerequisites for the disease:
1. Acceptance of certain medicines;Often, signs of Lyell and Stevens Johnson syndrome occur with an overdose of drugs. There are cases of illness due to taking just one and the correct dose of the drug. Most cases of the disease were provoked by taking antibiotics, more often when taking antibiotics from the penicillin series.
A lesser likelihood of the disease is present when taking local anesthetics, sulfa drugs, nonsteroidal drugs anti-inflammatory purpose.
2. Infectious diseases;
Increased risk factors are bacterial and fungal infections. An acute allergic reaction to SJS can develop after exposure to herpes, HIV, influenza, and hepatitis viruses.
3. Oncological diseases;
4. Idiopathic form.
This is a disease whose causes have not been identified. Lyell and Stevens Johnson syndrome can occur for an unknown reason.
The main symptoms of SJS
In most cases, the syndrome begins to manifest itself as a sharp pain in the joints. SJS is an acute or rapid form of allergy so it starts unexpectedly.The patient may at first think that he is suffering from respiratory disease. At this stage, there is a strong persistent weakness, joint pain, fever. A sick person may suffer from nausea and vomiting. The initial state of the syndrome lasts several hours or several days. Then severe rashes appear on the mucous membranes and skin.
The rash appears in various places. A feature of the syndrome is symmetrical rashes. Allergic reaction takes its toll severe itching and burning sensation.
The rash is localized in different ways. In most patients, rashes are observed in the face, back of the hands and feet, on the knee and elbow folds. Mucous membranes in the mouth are more often affected and a little less often - the eyes.
The syndrome of Lyell and Stevens Johnson is defined by a rash in the form of papules, with a diameter of two to five millimeters. Each papule is visually divided into two parts. In the center of the vesicle, a small cavity with blood (hemorrhagic contents) and proteinaceous fluid (serous substance) is clearly defined. The outer part of the bubble has a bright red color.
Papules that form on mucous membranes are more painful. They quickly burst, leaving painful erosions with a yellow, unhealthy coating in place.
Mucosal damage in intimate places can cause urethral stricture in men and vaginitis in women.
When the eyes are affected, blepharoconjunctivitis and other eye diseases that impair vision develop.
With any form of Lyell's and Stevens Johnson's syndrome, patients feel pain and a growing sense of anxiety. Soreness with a rash on the mucous membranes leads to the refusal of food.
Diagnosis of the SJS syndrome
A detailed and correct medical history must be taken. SJS is one of the severe forms of an allergic reaction, so some patterns can be identified in the early allergic manifestations in a sick person. The patient should provide the attending physician with the most complete information - to tell if allergic reactions have happened before. Also, the doctor should know which substances and how severe the allergy was.The attending physician prescribes to the patient general analysis blood, biochemical research. The results of the tests will reveal the enzymes of aminotransferases, bilirubin and urea in the blood.
Through a thorough external examination, the strength and nature of the allergic lesion is revealed. Often a correct diagnosis is impossible without an immunogram. This study is aimed at searching for antibodies of a specific class in the blood.
By itself, the diagnosis of Lyell and Stevens Johnson syndrome is not a daunting task. Virtually all prescribed studies are designed to rule out pemphigus or other diseases with similar clinical manifestations.
How is SJS treated?
Identification of the syndrome of Lyell and Stevens Johnson requires urgent medical qualified assistance.
What urgently does the patient need before admission to the inpatient department? The following urgent actions are being taken:
Vein catheterization;
Infusion therapy (saline or colloidal solutions are introduced to reduce the concentration of allergens in the blood);
Intravenous administration of prednisolone in a single dose of 60-150 mg;
Due to severe edema of the mucous membrane of the larynx, the patient is transferred to a ventilator.
After placing the patient and stabilizing his condition in the hospital department, he is assigned the main treatment. The attending physician will prescribe analgesics for pain relief, glucocorticosteroid drugs against inflammation. When the skin or mucous membranes are infected, strong antibiotics are prescribed, except for preparations based on penicillin and vitamins.
Complications accompanying the syndrome of Lyell and Stevens Johnson require additional therapy.
Skin and mucosal lesions are treated antiseptic solutions and anti-inflammatory ointments.
The patient should receive a hypoallergenic diet, excluding the use of all types of fish, coffee, citrus fruits, honey and chocolate.
Stevens-Johnson syndrome is an acute bullous lesion of the mucous membranes and skin. People aged 20–40 years are susceptible to the disease, and it is extremely rarely diagnosed in infants under 3 years of age. Pathology is noted mainly in men. The syndrome is characterized by an acute course and the rapid development of complications with lesions internal organs. This necessitates the prompt provision of qualified assistance.
Causes
The main reason for the development of Stevens-Johnson syndrome, doctors call the use of medications. An acute allergic reaction is observed with an overdose of drugs or in case of individual intolerance to the components. As a rule, these are antibiotics of the penicillin group, non-steroidal anti-inflammatory drugs, CNS regulators, painkillers, sulfonamides and vitamins.
Rarely, the cause of Stevens-Johnson syndrome is an infectious disease. The infectious-allergic form occurs when it is affected by herpes, influenza, hepatitis or HIV, and in childhood The causative agent is the measles, mumps and chicken pox viruses. Sometimes a negative reaction is possible with fungal and bacterial infections.
An oncological disease (carcinoma or lymphoma) can provoke the syndrome. Sometimes doctors fail to establish the etiology of the disease, in which case they speak of an idiopathic form.
Symptoms
Stevens-Johnson syndrome is a lightning-fast allergic reaction that develops rapidly and is very acute. The first symptoms are similar to those of a respiratory disease. The patient develops weakness, fever, fever up to 40 ⁰С, joint pain, headache and sleepiness. There may be a sore throat or sore throat, dry cough.
In some cases, dyspeptic disorders are observed: nausea, vomiting, diarrhea and a complete lack of appetite. Cardiovascular disorders - tachycardia (rapid heartbeat) and accelerated heart rate.
This condition persists for several hours, and then a symptom characteristic of the syndrome appears - rashes on the skin and mucous membranes.
The rash can be localized on different parts of the body, but, as a rule, the rashes are symmetrical. Mostly an allergic reaction is observed at the knee and elbow bends, on the face and on the back of the hand and feet. The rash also occurs on the mucous membranes - in the mouth, on the eyes and genitals. The rash is accompanied by severe burning and itching.
Outwardly, the rash looks like papules with a diameter of 2–4 mm. In the center of the formation is a vial with serous or hemorrhagic fluid. The outer part of the papule is bright red. Bubbles localized on the mucous membranes quickly burst, leaving painful erosions in this place, which eventually become covered with a yellow coating.
The lesion of the mucous membrane of the eyes is similar to allergic conjunctivitis. Often a secondary infection joins, which causes an acute inflammatory process with purulent discharge. Erosive and ulcerative lesions are formed on the cornea and conjunctiva. Perhaps the development of keratitis, blepharitis or iridocyclitis.
With mucosal injury oral cavity and the red border of the lips, the patient has difficulty eating and drinking. Nutrition is provided through a probe, and medications are administered intravenously.
The psycho-emotional state of the patient is aggravated. He experiences anxiety and irritability, becomes withdrawn and apathetic. Due to constant itching and pain, sleep is disturbed, appetite worsens and performance decreases.
Diagnostics
To diagnose the syndrome, an anamnesis is taken. The doctor finds out whether the patient has a tendency to an allergic reaction, whether it has happened before and what served as its causative agent. It is important to detect the fact of taking medications or the presence of an infectious process. The doctor conducts a visual examination, assessing the condition of the skin and mucous membranes.
Laboratory diagnostic methods: general and biochemical blood tests. Diagnostic value is the level of urea, bilirubin and aminotransferase enzymes.
A coagulogram allows you to evaluate blood clotting and the rate of blood clots. An immunogram may be performed to detect specific antibodies in the blood. An elevated level of T-lymphocytes indicates the presence of pathology.
Sometimes when histological examination necrosis of epidermal cells is detected, perivascular infiltration by lymphocytes is diagnosed.
Instrumental diagnostic methods: CT of the kidneys, radiography of the lungs, ultrasound of the urinary system. In some cases, an additional consultation with a nephrologist, pulmonologist, urologist and ophthalmologist is required.
During diagnosis, it is important to differentiate Stevens-Johnson syndrome from pemphigus, Lyell's syndrome, and other pathologies that have similar symptoms.
Treatment
Stevens-Johnson syndrome requires immediate medical care. Before the patient is admitted to the hospital, it is important to carry out catheterization of the vein and start infusion therapy. To reduce the level of allergens in the blood, droppers with saline or colloidal solutions are used. Additionally, Prednisolone (60–150 mg) is administered intravenously to the patient. If swelling of the mucous membrane of the larynx develops, breathing is disturbed, then the patient is transferred to artificial ventilation of the lungs.
After the acute attack subsides, the patient is placed in a hospital, where he is constantly under the supervision of medical personnel. Analgesics are prescribed to relieve pain and relieve the condition. Glucocorticosteroids will help eliminate inflammation.
If necessary, intravenous transfusion of plasma and protein solution is performed. Additionally, drugs with a high content of calcium and potassium are prescribed. Used to fight allergies antihistamines- Suprastin, Diazolin or Loratadine.
In the event of a bacterial infection of the body, antibiotic therapy. At the same time, it is strictly forbidden to use antibiotics of the penicillin group and vitamin complexes. To improve the condition of the skin, anti-inflammatory ointments and antiseptic solutions are prescribed.
Forecast and prevention
With timely assistance, the prognosis is quite favorable. However, the syndrome is often accompanied by severe complications, which makes treatment difficult. As a rule, it is vaginitis in women and urethral stricture in men. With the defeat of the mucous eyes, blepharoconjunctivitis develops, visual acuity decreases. As a complication, the development of pneumonia, colitis, bronchiolitis and secondary infection is possible. Less commonly, acute renal failure develops and the process of producing hormones by the adrenal glands is disrupted. In 10% of cases, patients with Stevens-Johnson syndrome die.
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Stevens-Johnson Syndrome
What is Stevens-Johnson Syndrome -
Stevens-Johnson Syndrome(malignant exudative erythema) 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 diseases.
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 allergic reaction 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 sections of the eye (retina, etc.) may join.
The 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 genitalia). 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. Very pronounced violation general condition the patient, 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, kidney failure, 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 medicines. The external manifestations of the disease are assessed, 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 of immune complexes circulating in the blood, which are antibodies associated with antigens. To do this, special preparations are used for intravenous administration, methods of blood purification in the form of hemosorption and plasmapheresis.
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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Other diseases from the group Diseases of the skin and subcutaneous tissue:
| Abrasive pre-cancer cheilitis of Manganotti |
| actinic cheilitis |
| Allergic arteriolitis or Reiter's vasculitis |
| Allergic dermatitis |
| skin amyloidosis |
| Anhidrosis |
| Asteatosis, or sebostasis |
| Atheroma |
| Basalioma of the skin of the face |
| Basal cell skin cancer (basalioma) |
| Bartholinitis |
| White piedra (knotty trichosporia) |
| Warty skin tuberculosis |
| Bullous impetigo of newborns |
| Vesiculopustulosis |
| Freckles |
| Vitiligo |
| Vulvitis |
| Vulgar or strepto-staphylococcal impetigo |
| Generalized rubromycosis |
| Hydradenitis |
| Hyperhidrosis |
| Hypovitaminosis of vitamin B12 (cyanocobalamin) |
| Vitamin A hypovitaminosis (retinol) |
| Hypovitaminosis of vitamin B1 (thiamine) |
| Hypovitaminosis of vitamin B2 (riboflavin) |
| Hypovitaminosis of vitamin B3 (vitamin PP) |
| Vitamin B6 hypovitaminosis (pyridoxine) |
| Vitamin E hypovitaminosis (tocopherol) |
| hypotrichosis |
| Glandular cheilitis |
| Deep blastomycosis |
| Fungal mycosis |
| Epidermolysis bullosa group of diseases |
| Dermatitis |
| Dermatomyositis (polymyositis) |
| Dermatophytosis |
| splinters |
| Malignant granuloma of the face |
| Itching of the genitals |
| Excess hair, or hirsutism |
| Impetigo |
| Indurative (compacted) Bazin's erythema |
| True pemphigus |
| Ichthyosis and ichthyosis-like diseases |
| Skin calcification |
| Candidiasis |
| Carbuncle |
| Carbuncle |
| Pilonidal cyst |
| Skin itching |
| granuloma annulare |
| contact dermatitis |
| Hives |
| Red grainy nose |
| Lichen planus |
| Palmar and plantar hereditary erythema, or erythrosis (Lahn's disease) |
| Skin leishmaniasis (Borovsky's disease) |
| Lentigo |
| liveoadenitis |
| Lymphadenitis |
| Fusk line, or Andersen-True-Hackstausen syndrome |
| Lipoid necrobiosis of the skin |
| Lichenoid tuberculosis - lichen scrofulous |
| Riehl melanosis |
| skin melanoma |
| Melanoma dangerous nevi |
| Meteorological cheilitis |
| Mycosis of nails (onychomycosis) |
| Mycoses of the feet |
| Multimorphic exudative erythema |
| Mucinous alopecia of Pinkus, or follicular mucinosis |
| Hair growth disorders |
| Neacantholytic pemphigus, or scarring pemphigoid |
| Pigmentation incontinence, or flea-sulzberger syndrome |
| Neurodermatitis |
| Neurofibromatosis (Recklinghausen's disease) |
| Baldness or alopecia |
| Burn |
| burns |
| frostbite |
| frostbite |
| Papulonecrotic tuberculosis of the skin |
| Inguinal epidermophytosis |
| Periarteritis nodular |
| Pint |
| Pioallergides |
| pyoderma |
| pyoderma |
| Squamous cell skin cancer |
| Superficial mycosis |
| tardive cutaneous porphyria |
| Polymorphic dermal angiitis |
| Porfiria |
| graying hair |
| Scabies |
| Occupational skin diseases |
| Manifestation of vitamin A hypervitaminosis on the skin |
| The manifestation of hypovitaminosis of vitamin C on the skin |
| Skin manifestations of herpes simplex |
| Broca's pseudopelade |
| Finger pseudofurunculosis in children |
| Psoriasis |
| Chronic pigmentary purpura |
| Spotted atrophy of the Pellizzari type |
| Rocky Mountain Spotted Fever |
| Rocky Mountain Spotted Fever |
| versicolor |
This is an acute bullous lesion of the mucous membranes and skin of an allergic nature. It proceeds against the background of a serious condition of the diseased with the involvement of the oral mucosa, eyes and urinary organs. Diagnosis of Stevens-Johnson syndrome includes a thorough examination of the patient, immunological study blood, skin biopsy, coagulogram. According to the indications, X-ray of the lungs, ultrasound Bladder, Ultrasound of the kidneys, biochemical analysis of urine, consultations of other specialists. Treatment is carried out by methods of extracorporeal hemocorrection, glucocorticoid and infusion therapy, antibacterial drugs.
ICD-10
L51.1 Bullous erythema multiforme
General information
Data on Stevens-Johnson syndrome were published in 1922. Over time, the syndrome was named after the authors who first described it. The disease is a severe variant of erythema multiforme exudative and has a second name - "malignant exudative erythema". Together with Lyell's syndrome, pemphigus, bullous variant of SLE, allergic contact dermatitis, Hailey-Hailey disease, and others, clinical dermatology classifies Stevens-Johnson syndrome as bullous dermatitis, common clinical symptom which is the formation of blisters on the skin and mucous membranes.
Stevens-Johnson syndrome occurs at any age, most often in persons 20-40 years old and extremely rare in the first 3 years of a child's life. According to various data, the prevalence of the syndrome per 1 million population ranges from 0.4 to 6 cases per year. Most authors note a higher incidence among men.
Causes
The development of Stevens-Johnson syndrome is due to an allergic reaction of an immediate type. There are 4 groups of factors that can provoke the onset of the disease: infectious agents, drugs, malignant diseases and unknown causes.
In childhood, Stevens-Johnson syndrome often occurs against the background of viral diseases: herpes simplex, viral hepatitis, adenovirus infection, measles, influenza, chicken pox, mumps. A provoking factor can be bacterial (salmonellosis, tuberculosis, yersiniosis, gonorrhea, mycoplasmosis, tularemia, brucellosis) and fungal (coccidioidomycosis, histoplasmosis, trichophytosis) infections.
In adults, Stevens-Johnson syndrome is usually due to medication or malignancy. Of the drugs, the role of the causative factor is primarily assigned to antibiotics, non-steroidal anti-inflammatory drugs, CNS regulators and sulfonamides. leading role among oncological diseases in the development of Stevens-Johnson syndrome, lymphomas and carcinomas play. If a specific etiological factor of the disease cannot be established, then they speak of the idiopathic Stevens-Johnson syndrome.
Symptoms
Stevens-Johnson syndrome is characterized by an acute onset with rapid development of symptoms. At the beginning, there is malaise, a rise in temperature to 40 ° C, headache, tachycardia, arthralgia and muscle pain. The patient may experience sore throat, cough, diarrhea, and vomiting. After a few hours (maximum after a day), rather large blisters appear on the oral mucosa. After their opening, extensive defects are formed on the mucosa, covered with white-gray or yellowish films and crusts of gore. The red border of the lips is involved in the pathological process. Due to severe mucosal damage in Stevens-Johnson syndrome, patients cannot eat or even drink.
Eye damage at the beginning proceeds according to the type of allergic conjunctivitis, but is often complicated by secondary infection with the development of purulent inflammation. For Stevens-Johnson syndrome, the formation of erosive-ulcerative elements of small size on the conjunctiva and cornea is typical. Possible damage to the iris, the development of blepharitis, iridocyclitis, keratitis.
Organ mucosal injury genitourinary system observed in half of the cases of Stevens-Johnson syndrome. It proceeds in the form of urethritis, balanoposthitis, vulvitis, vaginitis. Scarring of erosions and ulcers of the mucosa can lead to the formation of urethral stricture.
The skin lesion is represented by a large number of rounded raised elements resembling blisters. They are purple in color and reach a size of 3-5 cm. A feature of the elements of the skin rash in Stevens-Johnson syndrome is the appearance of serous or bloody blisters in their center. The opening of the blisters leads to the formation of bright red defects, covered with crusts. The favorite localization of the rash is the skin of the trunk and perineum.
The period of appearance of new rashes of Stevens-Johnson syndrome lasts approximately 2-3 weeks, the healing of ulcers occurs within 1.5 months. The disease can be complicated by bleeding from the bladder, pneumonia, bronchiolitis, colitis, acute renal failure, secondary bacterial infection, loss of vision. As a result of developed complications, about 10% of patients with Stevens-Johnson syndrome die.
Diagnostics
The clinician-dermatologist can diagnose Stevens-Johnson syndrome based on characteristic symptoms detected by careful dermatological examination. Questioning the patient allows you to determine the causative factor that caused the development of the disease. A skin biopsy helps confirm the diagnosis of Stevens-Johnson syndrome. Histological examination shows epidermal cell necrosis, perivascular lymphocyte infiltration, and subepidermal blistering.
In a clinical blood test, nonspecific signs of inflammation are determined, a coagulogram reveals clotting disorders, and a biochemical blood test shows a low protein content. The most valuable in terms of diagnosing Stevens-Johnson syndrome is an immunological blood test, which detects a significant increase in T-lymphocytes and specific antibodies.
The variety of forms of skin lesions requires their detailed classification, which allows us to attribute the existing current disease to a specific type and draw up the most effective treatment regimen. After all, some of the forms have not only a very unpleasant course for the patient, but can also pose a danger to his life.
And one of these varieties is malignant exudative eczema multiforme, called Stevens-Johnson syndrome, which has characteristic symptoms with damage to the upper layer of the epidermis and mucous membranes. Its course is accompanied by an active deterioration in the general condition of the patient, pronounced ulceration of the surfaces, which, in the absence of the necessary medicinal effect, can lead to serious complications for human health. In this article, we will talk about the differences between Lyell and Stevens-Johnson syndromes, whether it is possible to swim, as well as the causes and treatment of the disease.
Features of the disease
Stevens-Johnson syndrome has a very rapid development with a rapid aggravation of the characteristic symptoms, which has a sharp negative effect on the patient's health. Skin lesions are expressed in the appearance of a rash on its surface, which gradually deepens into the upper layer of the epidermis and causes the formation of clearly defined lesions. In this case, the patient feels a significant soreness of the affected areas of the skin, even with a slight mechanical effect on it.
- This condition can occur at almost any age, but it is most often observed in people who have reached the age of 40.
- But, according to doctors, today such a pathological condition has begun to occur at a younger age, as well as among infants.
- In men, Stevens-Johnson syndrome occurs with the same frequency as in women, while the symptoms of the disease are completely similar.
Like any other skin lesion, Stevens-Johnson syndrome responds faster to treatment when it is detected at its maximum. early stages. Therefore, timely treatment for examination allows you to draw up a more effective treatment regimen to prevent further aggravation. pathological condition the patient's skin.
Syndrome of Lyell and Stevens-Johnson (photo)
Classification
In medical practice, there is a division of this condition into several stages, depending on the degree of neglect of the disease.
- At the initial stage lesions of the skin in Stevens-Johnson syndrome are observed, which is accompanied by a deterioration in the general condition, the appearance and loss of orientation in space. Some patients experience diarrhea, digestive disorders. Along with this, at the first stage of the development of the disease, lesions begin to appear on the skin, characterized by an increase in its sensitivity. The duration of the first stage can be from several days to 2 weeks.
- At the second stage progression of Stevens-Johnson syndrome, the area of the affected skin areas increases, the hypersensitivity of the skin also increases. On the surface of the skin, a small rash first appears, then with serous contents, the patient develops thirst, and saliva production decreases. At the same time, characteristic manifestations are noted both on the surface of the skin and on the mucous membranes, mainly the genital organs and the oral cavity. In this case, the rashes have a symmetrical arrangement, and the duration of the second stage of the development of the disease is no more than 5 days.
- Third stage characterized by a general weakening of the patient's body, the skin and mucous membranes with lesions hurt, it manifests itself very,. In the absence of medical care or its insufficiency, a fatal outcome is likely.
This video will tell about the features and concept of Stevens-Johnson syndrome:
Causes of Stevens-Johnson Syndrome
There are a number of reasons that provoke the occurrence of Stevens-Johnson syndrome and its progression. Reasons that can cause this condition to occur include:
- infectious lesions of the body, which dramatically reduce the degree of efficiency of the immune system. Most often, this cause becomes the main impetus for the onset of Stevens-Johnson syndrome in children and infants when they the immune system send off;
- the use of certain drugs, one of which contains a significant amount of sulfidamines, also provokes the occurrence of Stevens-Johnson syndrome;
- lesions of the body of a malignant nature, which include AIDS;
- the idiopathic variant of the disease can develop as a result of psychological overstrain, nervous overload and depressive states of a long course.
Also, the causes of the development of Stevens-Johnson syndrome include a combination of these causes or a combination of them.
Symptoms
The most characteristic manifestations of the activation of Stevens-Johnson syndrome include the deterioration of the skin, which occurs very quickly starting from the second stage of the current pathological process. In this case, the following symptoms are noted:
- an increase in body temperature and a general weakening of the body, while in some parts of the body places with red spots are formed. The sizes of such areas can vary significantly, their localization is different. The spots may be single, then they begin to merge. The location of the spots is usually symmetrical;
- after a few hours (10-12), swellings form on the surface of such spots, the upper layer of the epidermis begins to exfoliate. A bubble forms inside the spot, in which the serous fluid is grayish in color. When such a bubble is opened, an affected area remains in its place, which has increased sensitivity and soreness;
- gradually, the process covers an increasing surface of the skin, in parallel, there is a significant deterioration in the patient's condition.
With the defeat of the mucous membranes, there is increased sensitivity, swelling of the tissues and them. When opening the resulting blisters, an exudate of a serous-bloody composition is released, as a result of which there is a rapid dehydration of the patient. After opening the blisters on the skin remain larger, the skin on them has a bright red color and increased sensitivity.
When Stevens-Johnson syndrome is detected, a gradual aggravation of the current condition is noted, the skin surface changes its appearance, even with a slight mechanical impact on it, significant pain is noted with the formation of significant erosions, while no blisters form on the skin. Body temperature continues to be elevated.
Diagnostics
Thanks to the diagnosis initial stages development of the disease, it becomes possible to improve the patient's condition as quickly as possible. For diagnostics, methods such as a biochemical and general blood test, urinalysis, coagulogram data, as well as a biopsy of the victim's skin particles are used.
Manifestations of this condition may resemble other types of skin eczema, therefore it is laboratory methods that will avoid errors in the diagnosis. It is required to distinguish between Stevens-Johnson syndrome and,.
Treatment
Treatment, assistance with the development of Stevens-Johnson syndrome should be carried out as soon as possible to prevent a significant aggravation of the pathological process, which allows the patient to save life.
First aid consists in replenishing the body of the victim with fluid, which he constantly loses in the process of activating pathological processes in the skin.
The video below will tell about the diagnosis and treatment of Stevens-Johnson syndrome:
Therapeutic way
Since this condition is characterized by a rapid aggravation of pathological processes in the skin, providing assistance in a therapeutic way does not have a pronounced effectiveness. The most effective is the intake of certain medications to relieve pain and eliminate the main symptoms of the disease.
Bed rest and a diet based on liquid and puree foods can be considered as an important therapeutic agent for this condition.
In a medical way
The most important at the stage of activation of the manifestation of Stevens-Johnson syndrome is the use of glucocorticosteroids. Also, medicines with a pronounced effect should include:
- cancellation of previously taken drugs to eliminate the possibility of exacerbation of the current condition;
- infusions to prevent severe dehydration;
- disinfection of the skin with the help of products that dry the affected areas;
- taking antibacterial drugs;
- antihistamines that relieve skin burning and itching;
- disinfection of mucous membranes with ointment or hydrogen peroxide.
Efficiency in the provision of medical care determines its effectiveness and obtaining pronounced results in the treatment.
Other Methods
- Surgery is not recommended for Stevens-Johnson syndrome.
- Alternative methods are also powerless in the active process of skin lesions.
Stevens-Johnson syndrome (photo of a child)
Disease prevention
As preventive measures, one can name the exclusion of bad habits, the preparation of a menu based on healthy food, regular examination by a doctor in order to identify any abnormalities and diseases.
Forecast
When starting treatment in the early stages, the survival rate is 95-98%, with more neglected - from 60 to 82%. In the absence of assistance, the patient dies in 93% of cases.
This video will talk about Stevens-Johnson syndrome in a young girl and about the fight against such an ailment: