Lyell syndrome is a serious illness.toxic-allergic nature, entailing an acute violation of the general condition of the patient, as well as characterized by bullous formations on the entire skin and mucous membranes. Synonymous names for this pathology: acute or toxic epidermal necrolysis.
Etiology
The definition of a disease indicates itstoxic and allergic etiology. What does all this mean? Inflammatory processes on the background of allergies cause the body toxins. There is a detachment of the surface layer of the skin with further necrotization.
The syndrome is considered a type of bullousdermatitis. Named in honor of the doctor Lyell, who in 1956 first described it as a severe form of toxodermia. The clinical picture can be compared with a skin burn of 2 degrees. Another common name for the syndrome is "malignant pemphigus."
Along with anaphylactic shock, this diseaseIt is considered the most severe allergic reaction. And it should be noted that in addition to this deadly pathology, there is another, somewhat similar to her disease, which is called Stevens-Johnson syndrome (abbreviated as MAS). They are often confused, but in the competence of experienced specialists the recognition of these ailments.
Causes of Lyell Syndrome
Some of the known pathological cases are caused byacute allergic reactions to drugs. The most common cause of sulfonamides. More rarely, tetracycline antibiotics, erythromycins, anticonvulsants, some painkillers, anti-tuberculosis and anti-inflammatory drugs. There are also isolated cases of individual allergic reactions to dietary supplements, vitamin preparations, as well as tetanus toxoid and radiopaque agents.
As another group of cases can be identifiedreactions to infectious processes, most often caused by Staphylococcus aureus group II. Such a severe allergy (Lyell syndrome, not Johnson syndrome) is usually observed in childhood and has an extremely severe course.
There are a number of idiopathic cases, the causes of which remain unknown.
The development of the syndrome was also observed in the background.therapy of severe forms of various pathologies. There is an assumption that it is a combination of factors, infectious and drug, is the cause of the development of this disease.
Pathogenesis
The described pathology manifests itself quite quickly: from several hours to 1 week. There are cases of its development and at a later date.
It is believed that the occurrence of the syndromeLyell is provoked by an individual's increased allergic sensitivity of the body, which is a genetically determined trait. This is a kind of severe drug intolerance.
The history of many patients contains allergic manifestations such as rhinitis, pollinosis, contact dermatitis, eczema, asthma, etc.
The mechanism of development of an exotic reaction
How does Lyell syndrome in children?The body disrupts the process of neutralization of metabolic products. Thus, the drug combines with protein, which is part of the surface layers of the skin. As a result, a foreign substance is formed in the structure of the latter, to which an acute immune response follows. It turns out that the skin itself becomes the strongest allergen. This can be equated to the reactions of the body to a foreign transplant. Only in this case there is nothing stranger.
Interestingly, all patients with syndromes and Lyell and Johnson are characterized by the presence of immunological reactions in the body, known as the Schwartzman-Sanarelli phenomenon.
Disturbances in the body
In the body, the process of chains is disturbed.protein substances and regulation of their decay. This is manifested in their uncontrolled and very rapid cleavage, as a result of which their accumulation occurs in the body (in its fluids), which in turn has a toxic effect on the internal organs.
Все это ведет к тому, что и функции самих bodies that are responsible for the neutralization of degradation products and their conclusion, also suffer greatly. As a result, the water-salt balance in the body is disturbed, as well as the balance of mineral substances. The patient's condition is rapidly and rapidly deteriorating, and if emergency medical care is not provided in time, the probability of a fatal outcome is high. Mortality in Lyell syndrome is very high.
Symptoms and diagnostic features
The disease affects mainly young people and children. Diagnosis is usually not difficult, since the symptoms and simple laboratory indicators are clearly expressed in this case.
Above, we noted that the disease is characteristicdevelopment in a short time. The condition worsens dramatically, the patient may even die before the start of treatment. It all starts with a sharp rise in temperature, sometimes to critical values, the body is covered with abundant rashes in the form of red spots with small edema. Diagnosis of Lyell syndrome should be done very quickly.
Increase in spots in size
Gradually there is an increase in spots in size, they merge together, forming large lesions. Then the process becomes similar to the picture of the development of burns of II degree:
- After about 2 days, the affected areas become covered with blisters of various sizes. The skin on them is very thin and subject to mechanical injury, it is very easy to tear.
- After some time, the sight of the patient becomes as if he was scalded with boiling water.
- The skin is extremely painful, there is a symptom of "wet linen": when you touch it, it is easy to move into the fold. It is also possible the appearance of an abundance of minor hemorrhages throughout the body.
Mucous membranes suffer
В процессе и синдрома Стивенса-Джонсона, и Lyell syndrome suffer from mucous membranes. In the mouth there is the presence of defects that are extremely painful, even with a light touch. There is a high bleeding.
On the lips - the same picture. Later, the affected lesions are covered with crusts. The thickness and location of them are such that the patient can not eat.
Damage to the upper respiratory tract, gastrointestinal tract and even the bladder may be observed.
The death of the surface layer of the skin
Микроскопическое исследование материала выявляет the fact of the death of the entire surface layer of the skin, under which a significant number of blisters are found. Their contents are sterile, there are no pathogenic bacteria in it.
Deeper layers in Lyell's syndromediffer in edema, they reveal a huge number of immune cells that have invaded the bloodstream. They are located near the vessels, while the walls of the latter also swell.
System-wide signs of damage
As we have already noted, the general condition of the patient is rapidly deteriorating:
- There is a high temperature, severe headache, orientation in space can be disturbed, clouding of consciousness, drowsiness can occur.
- There are signs of dehydration in the form of strong thirst, dry mouth, a decrease in the amount of salivation.
- The work of the cardiovascular system is disturbed, the blood thickens, which consequently leads to failures in the work of all organs and the organism as a whole.
- Since the key point of this diseaseis a strong violation of water-salt balance, it is a source of significant dehydration, intoxication occurs with the products of the breakdown of organs and their tissues.
- Violation of protein synthesis and the mechanisms of their cleavage leads to the accumulation in the tissues of a huge number of smaller proteins.
Laboratory values
With Lyell's syndrome (in the photo you can seethe initial stage of the disease) a blood test shows high leukocytosis, which indicates an intense inflammatory process in the body. The leukocyte formula shows the appearance of immature forms of leukocytes, ESR is increased, blood coagulation is also higher than normal.
A biochemical blood test reveals significanta decrease in protein in it, but the content of protein bodies is high. Bilirubin is also increased, which is an indicator of impaired liver function. There is also a high content of urea, which indicates impaired renal function. There is a high nitrogen content and activity of some enzymes.
A urinalysis reveals a protein in it.
What are the recommendations for Lyell syndrome?
Treatment
Timely identification is very important.signs of the described illness, providing emergency medical care to the patient and placing him in a hospital, preferably immediately in the intensive care unit. We have already noted how much the patient's condition worsens, and what generalized consequences and complications this pathological process leads to.
Oddly enough, but the most critical period of the disease is 2-3 weeks, when the above complications develop. It is at this time that it is important to provide maximum assistance.
High mortality
Despite continuous improvementtherapeutic methods, with Lyell's syndrome, mortality remains quite high - approximately 30% of all cases die. The later the diagnosis is made and treatment is started, the higher the likelihood of death. A similar probability increases with damage to more than 50% of the skin.
Therapeutic methods for treating Lyell syndromeaimed at combating pathological changes in the body, as a result of the toxic effect of the decay products of proteins and tissues of organs. They also support the water-salt and mineral balance in the body, and measures are being taken to reduce blood coagulation.
Intensive Care Techniques
With symptoms of Lyell's syndrome, intensive care is performed. It usually looks like this:
- Extracorporeal hemosorption.This is method number 1 in the treatment of Lyell's syndrome. It is advisable to use it in the first two days, as further the effectiveness of therapy is markedly reduced. With timely initiation of treatment, 2-3 procedures are enough for the patient to recover completely. After a few days, the patient's condition worsens noticeably, and the toxic damage to the body is gaining momentum. In this case, more procedures may be required.
- Plasmapheresis It has bidirectional action.First, it removes toxins, allergens, immune complexes circulating in the blood, as well as active immune cells. The second - it normalizes the body's defense. At the initial stage of the disease, 2-3 procedures are also sufficient to restore the patient's normal condition.
- Therapeutic methods aimed atstabilization of water-salt and protein balance in the body. This is a very important milestone in treatment, since it is the mentioned violation that triggers the generalized pathological processes of internal organs. Liquid is constantly injected into the body (about 2 liters per day) in the form of special solutions for intravenous injection.
- Since during the period of the described disease isactive decay of the skin and its own organs, the patient is injected with special drugs that inhibit the production of enzymes aimed at destruction.
- Corticosteroid preparations. They have a pronounced anti-inflammatory and anti-allergic activity. Their doses during the intensive care period are increased.
- Drugs to ensure the normalization and maintenance of liver and kidney function.
- Antibiotics. Their goal is rather preventive.Since the body is undergoing active inflammatory and destructive processes, it is likely that infectious lesions will join. But the choice of antibiotics is very strict, since in this case they are dealing with a patient with a high degree of sensitivity to drugs and a pronounced allergic reaction.
- The intake of minerals at the same time as diuretics. These are drugs of potassium, calcium, magnesium.
- Medicines that help reduce blood coagulation.
- Wound healing is applied to skin lesionsointments, corticosteroid aerosols, local antibacterial drugs, a regular change in sterile dressings is performed. As wound healing and anti-inflammatory drugs, rinsing the oral cavity with decoctions of herbs of antibacterial significance is prescribed.
Treatment, as already mentioned, is carried out inintensive care wards, where they conduct intensive monitoring and provide proper patient care. Unacceptable cold in the room. Be sure to equip the premises with UV lamps of a bactericidal spectrum of action.
