Lyell's syndrome is a serious illness of a 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 of 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. Detachment of the surface layer of the skin with further necrotization occurs.
The syndrome is considered a type of bullous dermatitis. Named after the doctor Lyell, who in 1956 first described it as a severe form of toxicoderma. 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 ailment is considered the most severe allergic reaction. And it should be noted that in addition to this deadly pathology, there is another, somewhat similar disease, which is called Stevens-Johnson syndrome (abbreviated as SJS). They are often confused, but the competence of experienced specialists recognizes these ailments.
Causes of Lyell's Syndrome
Some known pathological cases are caused by acute allergic reactions to drugs. Most often, sulfonamides become the cause. Less commonly, 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 X-ray contrast agents.
As another group of cases, we can distinguish reactions to infectious processes, most often caused by Staphylococcus aureus II group. Such a severe allergy (Lyell syndrome, not Johnson's syndrome) is usually observed in childhood and has an extremely severe course.
There are a number of idiopathic cases, the causes of which remain undetermined.
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 at a later date.
It is believed that the onset of Lyell syndrome is provoked by an individual's increased allergic sensitivity of the body, which is a genetically determined sign. This is a kind of severe drug intolerance.
The history of many patients contains allergic manifestations such as rhinitis, hay fever, contact dermatitis, eczema, bronchial asthma, etc.
The mechanism of development of an exotic reaction
How does Lyell's syndrome manifest itself in children? The body disrupts the process of neutralizing metabolic products. 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 body's reactions 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 Schwarzman-Sanarelli phenomenon.
Disorders in the body
In this case, the process of chains of protein substances and the regulation of their breakdown are disrupted in the body. This is manifested in their uncontrolled and very fast splitting, resulting in their accumulation 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 for the neutralization of decomposition products and their removal, also suffer greatly. As a result, the water-salt balance in the body is disturbed, as well as the balance of minerals. The patient’s condition deteriorates sharply and quickly, and if emergency medical care is not provided on 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 in this case are clearly expressed.
We noted above that a disease is characterized by development in a short time. The condition worsens sharply, the patient may even die before 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's syndrome should be carried out very quickly.
Spots increase in size
Gradually, spots increase in size, they merge with each other, forming large foci of lesions. Then the process becomes similar to the picture of the development of burns of the II degree:
- After about 2 days, the affected areas are covered with blisters of various sizes. The skin on them is very thin and prone to mechanical injuries, it is very easy to tear.
- 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 clothes”: when touched, it is easy to move it into a crease. It is also possible the appearance of an abundance of minor hemorrhages throughout the body.
Slimy suffer
In the process, both Stevens-Johnson syndrome and Lyell syndrome suffer from mucous membranes. In the mouth there are defects that are extremely painful even with a light touch. There is high bleeding.
On the lips - the same picture. Later, the affected lesions become crusted. Their thickness and location are such that the patient can not eat.
Damage to the mucous membranes of the upper respiratory tract, gastrointestinal tract and even the bladder can be observed.
Death of the surface layer of the skin
Microscopic examination of the material reveals the fact of death of the entire surface layer of the skin, under which a significant number of bubbles are detected. Their contents are sterile, there are no pathogenic bacteria in it.
The deeper layers in Lyell's syndrome are characterized by swelling, 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 may be disturbed, clouding of consciousness, drowsiness occurs.
- There are signs of dehydration in the form of severe thirst, dry mouth, decreased salivation.
- The work of the cardiovascular system is disrupted, the blood thickens, which accordingly entails disruptions in the work of all organs and the body as a whole.
- Since the key point of this disease is a strong violation of the water-salt balance, this is a source of significant dehydration, intoxication occurs with the products of decay of organs and their tissues.
- Violation of protein synthesis and their cleavage mechanisms 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 high leukocytosis, which indicates an intense inflammatory process in the body. The leukocyte formula shows the appearance of immature forms of white blood cells, ESR is increased, blood coagulation is also higher than normal.
A biochemical blood test reveals a significant decrease in protein in it, but the protein body content is high. Bilirubin is also increased, which is an indicator of impaired liver function. There is also a high urea content, 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
It is very important to timely identify the signs of the described illness, 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 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 rate
Despite the continuous improvement of therapeutic methods, with Lyell's syndrome, mortality remains quite high - approximately 30% of all patients 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 of treating Lyell's syndrome are aimed at combating pathological changes in the body, as a result of the toxic effect of the breakdown 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 at stabilizing the 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 there is an active 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 are increased during intensive care.
- 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 ointments, corticosteroid aerosols, local antibacterial drugs are applied to the skin lesion area, and sterile dressings are regularly changed. 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 in intensive 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.