Lyell's syndrome (toxic epidermal necrolysis): treatment

Lyell's syndrome or toxic epidermal necrolysis is a severe polyetiological disease with allergic the nature and manifested by a sudden violation of the patient's condition, bullous lesions of the mucous membrane and surface layers skin.

Lyell's syndrome is sometimes associated with severe Stevens-Johnson syndrome. Numerous studies have revealed a similar pathogenetics of these forms of the disease. The differences between Lyell's syndrome include toxic lesions of the epithelium to certain groups of drugs or their metabolites. There is still no clear distinction between Stevens-Johnson syndrome and Lyell's syndrome.

Both of these conditions are severe malignant exudative erythema as a result of drug toxicidermy, characterized by severe dehydration, toxic damage to internal organs and the development of an infectious process, which is accompanied by syndrome. All these symptoms together and each separately can lead to death of the disease.

The mechanism of development of the disease

Lyell's syndrome develops due to antigens entering the patient's body from the outside. The most common allergens are drugs or microbial breakdown products. Pathogenic microorganisms are not able to be excreted due to the formed defect of the neutralizing system, as a result of which there is a fixation (connection) of proteins of the epidermal cell with antigens.

As a result of the development of the syndrome, the immune system turns on a protective mode in the form of antibodies directed to antigens. The consequence of this reaction is a necrotic change in epidermal cells. If we compare the pathogenesis of the disease, then it is very similar to the syndrome of rejection of donor organs with incompatibility. In this case, the patient's own skin plays the role of foreign tissue.

Factors contributing to the development of the disease

The main factor contributing to the appearance of Lyell's syndrome is the intolerance of patients to certain types of drugs in the treatment of diseases.

The most dangerous drugs include sulfonamides (Biseptol, Sulfodimethoxin, Streptocid, Sulfasalazine and Sulfalen). In addition, the following groups of drugs are capable of leading to the development of the syndrome:

• antibacterial drugs (penicillin, tetracycline and macrolides) and especially Erythromycin;
• anticonvulsants (Phenobarbital, Phenytoin);
• anti-inflammatory and pain relievers (Aspirin, Diclofenac, Analgin);
• anti-tuberculosis (Isoniazid);
• vitamins and contrast agents for radiography;
• Dietary supplements and immunopreparations.

One of the likely factors in the occurrence of Lyell's syndrome is an acute infectious process caused by staphylococcus. In addition, cases are described when the causes of Stevens-Johnson syndrome have not been clarified, i.e. at full the absence of provoking factors (infection, drugs, etc.), the pathological process, however, progressed.

Types of Lyell's syndrome

The type of syndrome is determined taking into account the etiology. The following forms of the syndrome stand out the most:

IDIOPATHIC. This group includes all cases with unclear reasons for the development of the symptom of epidermal toxic necrolysis.

MEDICINAL. This form of the disease develops as a result of drug exposure.

STAPHYLOGENIC. The causes of the disease are staphylococcal infection. This type of Lyell's syndrome can only occur in children. It does not depend on the use of drugs and is not fatal. As a rule, the prognosis for the recovery of this form of the disease is favorable in most cases.

OCCURRING WITH SECONDARY PATHOLOGIES. This group is defined by cases when symptoms appear against the background of psoriasis, chickenpox, pemphigus, shingles.

The main sites of localization of lesions are the abdomen, shoulders, chest, gluteal region, back and oral cavity.

Symptoms

The syndrome is most common in children and young patients. The acute period lasts from 5 hours to 2-3 days. During this time, the patient's condition deteriorates sharply, and in especially severe cases it can threaten the patient's life.

At the initial stage of the onset of Lyell's syndrome, the following symptoms are observed:

• there is a sharp hyperthermia of the body up to the highest possible numbers. Brown spots are formed on the surface of the skin (body, face and limbs), which are surrounded by edematous tissue. The spots are characterized by rapid drainage into one large inflammatory area that hurts when touched;
• then there is the appearance of a huge number of watery blisters, like burns (pictured). Watery blisters are large enough, some of which can be as large as the palm of an adult patient. After spontaneous opening, bleeding erosive ulcers are found at the site of the blisters;

• with a slight mechanical effect on the affected area, the epidermal layer of the skin begins to exfoliate, resembling wet linen. It is characteristic that when the upper skin layer peels off from the area of ​​the fingers and toes, it is able to maintain its original shape. In addition, with light pressure on the bubble, it increases in size and exfoliates along the periphery;
• the mucous membranes are covered with cracks and abrasions, which are accompanied by severe pain and bleeding at the slightest physical intervention. Particular discomfort to the patient is caused by crusts on the lips (pictured), which make it difficult to eat;

• Lyell's syndrome can appear not only on the epidermis and mucous membranes. Its characteristic manifestations are possible on the inner bronchial membrane, in the intestines, nasopharynx, in the larynx, on the esophagus, as well as in the urethra and ureter;
• destruction of skin and mucous tissues leads to a sharp deterioration in the patient's condition (pictured). Hyperthermia is accompanied by severe headaches and excruciating thirst;

• at this time, the patient becomes lethargic and inhibited. Massive fluid loss leads to blood clotting. This complicates the functionality of the whole organism. Toxins from the breakdown of the epidermis have a negative effect on brain activity, leading to spatial disorientation and impaired behavioral activity.

It is important to note that the damaged surfaces that form during the syndrome are open wounds that are quite often become infected and are accompanied by purulent inflammatory complications, which poses a real danger to life. In addition, there is a blockage of blood vessels, which leads to impaired renal function.

These symptoms are characteristic, therefore, they are checked as a matter of priority when the doctor suspects that this is Lyell's syndrome.

Diagnostic examination

To determine the Stevens-Johnson syndrome, an external examination of the patient is carried out, and the characteristic symptoms of the disease are determined.

To differentiate from some other diseases with similar symptoms, laboratory blood tests can be performed (leukocytosis is determined). In addition, the characteristic differences of Lyell's syndrome are low eosinophilic counts or their absolute absence.

In addition, a blood test determines a decrease in the number of leukocytes and an increase in stab and neutrophils. The proteinogram notes a low total protein level and an increase in globulins.

Liver tests, which are carried out with the development of the syndrome, reveal an increase in all indicators, and a renal test is characterized by an increase in urea and nitrogen levels. At the same time, the electrolyte balance is sharply disturbed.

Lyell's syndrome (epidermal necrolysis) must be differentiated from "burnt skin" syndrome, which is most often observed among adult patients in the form of a reaction to the adoption of various medicines. In some cases, there is a lethal outcome of the disease.

To perform differential diagnosis, it is recommended to carry out a puncture with the collection of the necessary material for a biopsy, followed by examination of the skin section. As a rule, in Lyell's syndrome, the entire epidermis is exfoliated, in contrast to the “burnt skin” syndrome, when only the stratum corneum is exfoliated.

In order to clarify the diagnosis, an immunological test may be prescribed, when a provoking drug causes active multiplication of immune cells. It is important to distinguish Lyell's syndrome from similar diseases with similar symptoms, for example, bullous forms of multimorphic exudative erythema.

Quite often, the attending physician is faced with the question of finding out the drug that led to the development of the disease, because for the provision of emergency care, you have to use various types of medicinal funds. Therefore, it must be remembered that the use of a drug that provoked the consequences of an allergy in a patient can lead to serious complications.

The course of the disease in newborns

As a rule, newborn children are faced with the negative influence of the external and internal environment, which has a strong effect on the immature body, provoking the development of the disease. Of course, the pathogenesis of Lyell's syndrome in childhood has a serious impact on the general condition of the child and further development.

It should be noted that the immunity received from the mother, which protects the newborn baby for a certain period of time, is of no small importance. It is for this reason that Lyell's syndrome is rarely diagnosed in newborn babies. This condition can develop only against the background of staphylococcal infection.

In this case, in the formation of the disease, the main role belongs to the allergic-hereditary preparedness of the baby's body. Numerous observations of the course of the disease during this period of child development indicate the rapid development of symptoms in children, accompanied by general intoxication of the body. In addition, damage to the skin may well lead to the development of sepsis.

However, you need to know that with timely treatment started, the prognosis for recovery in babies is twice as high as in adult patients. Therefore, an important condition is to seek highly qualified medical care in the event of primary clinical signs of the disease so that professional treatment of Lyell's syndrome can prevent further complications.

Treatment tactics

All therapeutic measures for the development of the syndrome are mandatory only in a hospital setting (in burn departments or intensive care units). First of all, all drugs that can cause Lyell's syndrome are canceled. Next, the patient should be undressed and put into a special bed, maintaining a normal temperature and blowing the patient's body with warm jets of sterile air.

Erosive surfaces are treated with open methods using sterile dressings. In addition, it is recommended to treat the skin with external agents with hormones and antibiotics (Prednisolone, Methylprednisolone, Gordox, Contrikal, etc.). This is necessary to prevent the spread of secondary infection in the syndrome.

To compensate for dehydration during the development of the syndrome, it is recommended to give the patient a lot of fluid, and See also Oralit and Rehydron (rehydration recovery agents), taking into account the electrolyte level blood. Any liquid should be taken only warm. If it is impossible to take it on its own (unconsciousness), the introduction of nutrient mixtures through a tube is required. In case of complications of the disease, the required amount of nutrient solutions is administered by infusion, taking into account hematocrit and diuresis.

To get rid of toxins that are actively destroying the epidermal tissue, hemosorption is performed, when the blood is purified using a special filter and returned to the patient. In addition, a plasmapheresis procedure is performed, which involves the collection of a certain amount of blood from which the plasma is removed and the erythrocyte mass remains. After cleansing, it returns to the patient. The remaining plasma is replaced with a solution. In the event that this procedure is carried out within the first two days, there is the possibility of a quick recovery from the syndrome. With an increase in the duration of the procedure, the risk of a longer treatment of the disease increases.

It is important to note that Lyell's syndrome cannot be cured with traditional medicine. In order to alleviate the patient's condition, you can use various rinses (sage, chamomile, etc.) of the oral cavity. In addition, the affected areas can be lubricated with whipped egg white, and in the absence of allergies, it is recommended to treat these areas with vitamin A, which prevents cracking of the skin.

Complications of the disease

Quite often, the infectious consequences of the development of Lyell's syndrome develop, which is provoked extensive damage to the epidermis and the possibility of free penetration of pathogenic microbes into the wound surface. Most often, pneumonia, pyoderma and, the most dangerous disease, sepsis develop.

Due to the loss of a huge amount of moisture during an acute attack of the syndrome, along with which from the body salt and proteins are washed out, there is a thickening of the blood, which leads to a decrease in the blood supply to the internal systems and organs.

There is a deterioration in cardiac activity and respiratory failure. Stopping filtration of the kidneys and subsequent poisoning with toxins are possible. In addition, as a result of blood thickening, there is a high probability of blood clots forming in the pulmonary system and cerebral circulation, which leads to heart attacks and strokes.

Due to damage to the mucous membrane of the stomach and intestines, the absorption of nutrients may deteriorate and the likelihood of extensive internal bleeding is possible.

In children, Stevens-Johnson syndrome is characterized by a lightning-fast increase in negative symptoms, which is expressed by a sharp dehydration of the body and the development of infectious-toxic shock.

With serious lesions of the conjunctiva, the development of temporary blindness is likely. After the treatment of the syndrome, the patient may experience photophobia for a long period of time.

As a rule, the prognosis for recovery, when the diagnosis of Lyell's syndrome is confirmed, is quite serious. In percentage terms, mortality rates among such patients range between 35 and 70%. Most often, mortality is observed among children and elderly patients. The reasons for this are in a weakened body. Most often, death occurs from the development of sepsis, dehydration and thromboembolism.

Prevention of the development of Lyell's syndrome

Preventive measures to prevent the disease consist in strict adherence to the dosage of medicines prescribed by the doctor.

1. When seeking medical help, the patient must indicate to the attending physician all inadequate reactions of the body to any drugs and substances.
2. It is strictly forbidden to take more than 5-6 different medications at the same time (if this is not provided for by specific treatment).
3. It is important to remember that self-medication of patients (including prescriptions of traditional medicine), predisposed to various types of allergic reactions, can lead to the development of Stevens-Johnson syndrome. In this case, the prognosis for full recovery is dubious.
4. It should be noted that toxic epidermal necrolysis is a rather serious pathology that requires mandatory medical intervention. The lethality of the disease in intensive care conditions can reach 70%.

Currently, there is no universal cure for a complete cure for Stevens-Johnson syndrome, but the early treatment of Lyell's syndrome allows you to keep the disease under the supervision of specialists, significantly reducing the clinical symptomatology. The earlier treatment measures are started and the more thoroughly all treatment requirements are met, the more favorable the prognosis. This allows you to achieve long-term remission of the disease and prevent possible negative complications.