Lyell's syndrome is a serious disease of a toxic-allergic nature, which entails an acute violation of the general condition of the patient, and is also 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 the disease indicates its toxic-allergic etiology. What does all this mean? Inflammatory processes against the background of the manifestation of allergies cause damage to the body by toxins. There is a detachment of the surface layer of the skin with further necrosis.
The syndrome is considered a type of bullous dermatitis. Named after the physician Lyell, who first described it in 1956 as a severe form of toxicoderma. The clinical picture can be compared with a skin burn of the 2nd degree. Another common name for the syndrome is "malignant pemphigus".
Along with anaphylactic shock, this disease is considered the most severe allergic reaction. AndIt should be noted that in addition to this deadly pathology, there is another disease somewhat similar to it, which is called the Stevens-Johnson syndrome (abbreviated as SJS). They are often confused, but it is up to experienced professionals to recognize these ailments.
Causes of Lyell's syndrome
Some of the known pathological cases are caused by acute allergic reactions to drugs. The most common cause is sulfonamides. Less often - 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, reactions to infectious processes, most often caused by group II Staphylococcus aureus, can be distinguished. Such a severe allergy (Lyell's syndrome, not Johnson's) is usually observed in childhood and has an extremely severe course.
There are a number of other idiopathic cases, the causes of which remain unidentified.
The development of the syndrome was also observed during the treatment of severe forms of various pathologies. There is an assumption that it is a combination of factors, infectious and medicinal, that 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 inlater dates.
It is generally accepted that the occurrence of Lyell's syndrome is provoked by an individual increased allergic sensitivity of the body, which is a genetically determined trait. It's a kind of severe drug intolerance.
The anamnesis of many patients contains allergic manifestations such as rhinitis, hay fever, contact dermatitis, eczema, bronchial asthma, etc.
Mechanism for the development of an exotic reaction
How does Lyell's syndrome manifest itself in children? The process of detoxification of metabolic products is disrupted in the body. Thus, the drug combines with a protein that 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 reaction follows. It turns out that the skin itself becomes the strongest allergen. This can be equated with the reactions of the body to a foreign transplant. Only in this case, there is nothing alien.
Interestingly, all patients with both Lyell and Johnson syndromes 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 of protein substances and the regulation of their decay are disrupted. This is manifested in their uncontrolled and very rapid breakdown, as a result of which they accumulate in the body (in its fluids), which in turn has a toxic effect on internal organs.
All this leads to the fact that the functions of the organs themselves, which are responsible fordisposal of decay products and their withdrawal also suffer greatly. As a result, the water-s alt balance in the body is disturbed, as well as the balance of minerals. The patient's condition deteriorates sharply and rapidly, and if emergency medical care is not provided to him in time, then the probability of a fatal outcome is high. Mortality in Lyell's 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 parameters are clearly expressed in this case.
Above we noted that the disease is characterized by development in a short time. The condition deteriorates sharply, 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 profuse rashes in the form of red spots with small edema. Diagnosis of Lyell's syndrome should be carried out very quickly.
Increasing spots in size
Gradually, the spots increase in size, they merge with each other, forming large lesions. Then the process becomes similar to the picture of the development of burns of the second degree:
- About 2 days later, the affected areas are covered with blisters of various sizes. The skin on them is very thin and prone to mechanical injury, it breaks very easily.
- After a while, the patient's appearance becomes as if he had been scalded with boiling water.
- The skin is extremely painful, there is a symptom of "wet linen": when you touch iteasy to fold. There may also be an abundance of minor hemorrhages throughout the body.
Mucous sufferers
In the process of both Stevens-Johnson syndrome and Lyell's syndrome, mucous membranes suffer. In the mouth, there are defects that are extremely painful even with a light touch. High bleeding present.
On the lips - the same picture. Later, the affected lesions are covered with crusts. Their thickness and location are such that the patient cannot eat.
There may be damage to the mucous membranes of the upper respiratory tract, gastrointestinal tract and even the bladder.
Death of the surface layer of the skin
Microscopic examination of the material reveals 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.
The deeper layers in Lyell's syndrome are edematous, they reveal a huge amount of immune cells that have invaded from 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 may be disturbed, clouding of consciousness, drowsiness occurs.
- Show signs of dehydration in the form of extreme thirst, dry mouth, decreased salivation.
- The work of the cardio-vascular system, the blood thickens, which accordingly entails malfunctions in the work of all organs and the body as a whole.
- Since the key point of this disease is a severe violation of the water-s alt balance, this is a source of significant dehydration, intoxication occurs with the decay products of organs and their tissues.
- Violation of the synthesis of proteins and the mechanisms of their breakdown leads to the accumulation in the tissues of a huge number of smaller proteins.
Laboratory indicators
With Lyell's syndrome (in the photo you can see the initial stage of the disease), a blood test shows a 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 clotting is also above normal.
A biochemical blood test reveals a significant 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 a violation of kidney function. There is a high content of nitrogen and the activity of some enzymes.
Urine test detects protein in it.
What are the recommendations for Lyell's syndrome?
Treatment
It is very important to timely identify the signs of the described disease, provide emergency medical care to the patient and place him in a hospital, preferably immediately in the intensive care unit. We have already noted how muchthe 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 the constant improvement of therapeutic methods, with Lyell's syndrome, mortality remains quite high - approximately 30% of all cases die. The later the diagnosis is made and treatment started, the higher the chance of death. A similar probability increases with the defeat of more than 50% of the skin.
Therapeutic methods for the treatment of Lyell's syndrome are aimed at combating pathological changes in the body, as a result of the toxic effect of the decay products of proteins and organ tissues. The water-s alt and mineral balance in the body is also supported, measures are being taken to reduce blood clotting.
Intensive Care Techniques
With the symptoms of Lyell's syndrome, intensive care is carried out. It usually looks like this:
- Extracorporeal hemosorption. This is the number 1 method in the treatment of Lyell's syndrome. It is advisable to use it in the first two days, since further the effectiveness of therapy is markedly reduced. With timely treatment, 2-3 procedures are enough for the patient to fully recover. A few days later, the patient's condition noticeably worsens, and the toxic damage to the body is gainingturns. In this case, more treatments may be required.
- Plasmapheresis. It has a bidirectional effect. First, it removes toxins, allergens, immune complexes circulating in the blood, as well as active immune cells from the body. Second - it normalizes the body's defenses. At the initial stage of the disease, 2-3 procedures are also sufficient to restore the patient's normal state.
- Therapeutic methods aimed at stabilizing the water-s alt and protein balance in the body. This is a very important milestone in the treatment, since it is the mentioned violation that triggers the generalized pathological processes of the internal organs. The body is constantly injected with liquid (about 2 liters per day) in the form of special solutions for intravenous injections.
- Since during the period of the described disease there is an active decay of the skin and its own organs, the patient is given special drugs that suppress the production of enzymes aimed at destruction.
- Corticosteroid drugs. 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 purpose is rather preventive. Since active inflammatory and destructive processes are going on in the body, there is a high probability of attaching infectious lesions. But the choice of antibiotics is very strict, since in this case we are dealing with a patient with a high degree of sensitivity to drugs and brightsevere allergic reaction.
- Intake of minerals along with diuretics. These are preparations of potassium, calcium, magnesium.
- Medicines that help reduce blood clotting.
- Wound-healing ointments, corticosteroid aerosols, local antibacterial drugs are applied to the area of skin lesions, and sterile dressings are regularly changed. As a wound healing and anti-inflammatory agent, rinsing the mouth with decoctions of herbs of antibacterial value is prescribed.
Treatment, as already mentioned, is carried out in intensive care units, where they carry out enhanced monitoring and provide proper care for the patient. Cold is unacceptable in the ward. It is mandatory to equip the premises with bactericidal UV lamps.
