Lyell ‘s syndrome
Lyell’s syndrome (acute, toxic epidermal necrolysis) is a severe polyethological disease characterized by an allergic nature. This disease is characterized by acute impairment of the patient’s well-being, toxic damage to the kidneys and possibly other internal organs and organ systems.
The content of the article:
Causes of Lyell’s syndrome
The mechanism of development of Lyell’s syndrome
Symptoms of Lyell’s syndrome
Diagnosis of Lyell’s syndrome
Treatment of Lyell’s syndrome
Prognosis for Lyell’s syndrome
Lyell ‘s syndrome
In case of infection, this disease can lead to a fatal outcome. If we talk about the diagnosis of the disease, it involves examining the patient, monitoring the coagulogram, conducting clinical blood and urine tests. Treatment of Lyell’s syndrome is the implementation of timely measures, as well as methods of extracorporeal blood cleansing, infusion therapy, the use of significant doses of prednisone, the practice of antibiotic therapy, correction of water-salt deviations.
Lyell’s syndrome is attributed to bullous dermatitis. The name of the disease was given on behalf of the doctor Lyell, who described the syndrome in 1956. Then he described the disease as a severe form of toxicoderma. The clinic of the syndrome is very similar to a second-degree skin burn, which is why the disease is often confused with a burn skin syndrome. Another common name for the syndrome is malignant pemphigus. The latter is so called because of the presence of skin blisters, which look like particles of pemphigus.
Lyell’s syndrome occurs only in 0.5% of cases of allergic diseases. Like anaphylactic shock, Lyell’s syndrome is considered a severe allergic reaction. Very often Lyell’s syndrome occurs in young people, as well as in children. Symptoms of the disease appear after a couple of hours or for a week after the use of the medication. The mortality rate of Lyell’s syndrome is somewhere 30-50%.
Causes of Lyell’s syndrome
Regarding the causes of the development of Lyell’s syndrome in modern dermatology, 4 variants of the disease are determined.
The first option
The first option is an allergenic reaction that occurs as a response to an infectious process. This reaction is often explained by the presence of Staphylococcus aureus belonging to group 2. The first variant is observed in children and is characterized by its severe course.
The second option
The second option is Lyell’s syndrome, observed due to the use of medications (such as antibiotics, anti-inflammatory, sulfonamides, anti-inflammatory, anti-inflammatory, anti-tuberculosis). Very often, the formation of the syndrome is explained by the use of several drugs at once, including sulfonamide. Recently, there have been cases of the spread of Lyell’s syndrome with the use of biologically active additives, as well as vitamins and substances intended for radiography.
The third option
Another, the third variant of Lyell’s syndrome, includes ideopathic cases of the disease, the cause of which has not yet been clarified.
The fourth option
The fourth variant of Lyell’s syndrome is caused by various mixed causes (medicinal and infectious), usually develops against the background of treatment of an infectious disease.
The mechanism of development of Lyell’s syndrome
A significant role in the development of Lyell’s syndrome is assigned to the genetic component, which is due to a predisposition to allergic reactions. The anamnesis of most patients has indications of various allergic diseases, such as:
pollinosis;
eczema;
allergic rhinitis;
contact dermatitis of an allergic nature;
bronchial asthma.
In people suffering from this disease, the process of neutralization of various kinds of toxic metabolic products is disrupted, the injected medicinal components and the protein contained in the epidermis react. Such a newly formed substance is called an antigen characteristic of Lyell’s syndrome. That is, the immune response of the body is directed in response not only to the injected substance, but also to the patient’s dermis. This process is very similar to the reaction in which graft rejection occurs. It is known that with such a reaction, the immune system takes the patient’s skin itself for a transplant.
The basis of Lyell’s syndrome is the Schwarzman-Sanarelli phenomenon. This phenomenon is an immunological reaction that leads to failures in the regulation of the possibility of protein breakdown, as well as to the accumulation of products of this decay in the human body. As a result, toxic damage to organs and systems of human organs. The latter leads to disruption of the organs that are responsible for the neutralization and elimination of toxins, which means that the process is aggravated by intoxication, entails obvious changes in the water-salt balance (as well as electrolyte). Such processes lead to a deterioration of the patient’s condition in the case of Lyell’s syndrome. They can cause a fatal outcome.
Symptoms of Lyell’s syndrome
Usually Lyell’s syndrome begins unexpectedly with a suddenly elevated temperature (up to 39-40 ° C). Also, in just a few hours, small puffiness and erythematous spots appear on the patient’s skin, namely on the skin of the torso, oral mucosa, genitals, limbs and on the face, which are characterized by soreness. Such spots can be of very different sizes. Sometimes they merge.
After 12 hours, the epidermis begins to peel off on healthy skin. At this point, thin-walled bubbles of a sluggish nature and irregular shape appear. Their sizes reach the size of a hazelnut (up to 15 cm in diameter). After the opening of such bubbles, large erosions usually remain, which along their periphery are covered with fragments of the remnants of bubbles. Erosions are usually surrounded by edematous skin, which can also be hyperemic. Erosions secrete exudate, characterized by its abundant serous-bloody contents, which is the cause of instant dehydration of a person.
In the case of Lyell’s syndrome, the Nikolsky symptom inherent in pemphigus is noted, that is, the characteristic exfoliation of the epidermis due to the small effect on the skin surface. Due to the detachment of the epidermis, those areas that have undergone compression, as well as friction (or maceration), form erosion, while without the formation of bubbles.
Manifestations on the skin
With the course of this disease, the entire skin quickly turns red and a person feels a characteristic pain when touched. The appearance of such skin is very similar to a burn of 2 or 3 degrees. There is also a characteristic symptom when, during contact, the skin quickly wrinkles and shifts to the side. Sometimes manifestations of Lyell’s syndrome are accompanied by the appearance of small petechial rashes throughout the body. The main symptom of the disease in children is conjunctivitis, which is combined with infectious lesions of the dermis with a special staphylococcal flora.
Manifestation on mucous membranes
Damage to the mucous membranes is expressed by the appearance of painful defects on the skin, which usually bleed even in the case of minor injury. Such a process can affect the lips, mouth, mucous membrane of the eyes, larynx, trachea, bronchi, bladder, urethra, intestines and stomach.
Other symptoms
The general condition of patients with this syndrome can deteriorate dramatically and worsen even more after a while. Reduced sweating, extreme thirst, lack of saliva — all these are signs of dehydration in the body. Patients usually complain of a characteristic severe headache, may lose orientation and become drowsy. People with Lyell’s syndrome are characterized by hair and nail loss. Dehydration contributes to blood thickening and changes in the blood supply to internal organs. Along with toxic damage to the body, there is a violation of the liver, lungs, heart and kidneys. Anuria and severe renal failure may also develop. It is also possible that a secondary infection may be added.
Diagnosis of Lyell’s syndrome
In Lyell’s syndrome, a clinical blood test will usually indicate an inflammatory process. There is also an increased ESR, as well as leukocytosis with the appearance of immature forms. A reduced level of eosinophils (or their complete absence) in a blood test is considered a sign that helps distinguish Lyell’s syndrome from any other allergic conditions. The data of the performed coagulogram may indicate an increased level of blood clotting. A urine test and a biochemical blood test will help to detect disorders occurring in the tissues of the kidneys, as well as monitor the well-being of the body during treatment.
Before prescribing treatment, doctors suggest first determining the medication that could lead to the occurrence of Lyell’s syndrome, since its repeated use during therapy can be dangerous for the patient himself. To determine the substance that has become a provocateur, many immunological texts will help. The detection of a provoking drug can be indicated by the accelerated reproduction of immune cells, which occurs as a reaction to its introduction into a sample of the patient’s blood taken.
A biopsy or histological examination of a skin sample of a patient with Lyell’s syndrome can detect complete cell death of the epidermis surface. In the deep layers, the formation of large blisters, swelling and accumulation of immune cells with the highest concentration in the area of skin vessels can be observed.
Lyell’s syndrome is usually differentiated with acute forms of dermatitis, which are accompanied by the appearance of blisters:
pemphigus;
Stephen-Johnson syndrome;
actinic dermatitis;
contact dermatitis;
epidermolysis bullosa;
herpes simplex;
During’s herpetiform dermatitis.
Treatment of Lyell’s syndrome
Treatment of patients with Lyell’s syndrome is carried out in the intensive care unit. Treatment includes a whole range of different emergency medicine measures. Doctors take into account the toxic-allergic nature of the disease itself, medication should be taken strictly according to clinical indications or contraindications.
Lyell’s syndrome is treated by injection — through the introduction of significant doses of corticosteroids. In case of well-being, the patient is transferred to the drug already in tablet form with a gradual dose reduction. The use of extracorporeal hemocorrection, including hemosorption, plasmapheresis helps to purify the blood from the toxic substances that have arisen. Regular infusion therapy (hemodesis, physical solution, rheopolyglucin) is aimed at combating dehydration and normalizing the water-salt balance. Infusion therapy is carried out exclusively with strict control of the excreted urine.
For the complex therapy of Lyell’s syndrome, drugs are used that support the functioning of the liver and kidneys, as well as enzyme inhibitors that can participate in the destruction of tissues, all kinds of minerals (including magnesium, potassium and calcium), medications that reduce clotting, diuretics and broad-spectrum antibiotics.
The use of local drugs
If we talk about the local treatment of Lyell’s syndrome, it implies the use of aerosols with the presence of corticosteroids, the use of wet and fast-drying dressings, all kinds of antibacterial lotions. Local therapy is carried out in accordance with the rules of treatment of burns. In Lyell’s syndrome, to prevent infection, underwear is changed several times a day, skin and mucous membranes are treated. Taking into account the painfulness of the procedures, therapy is carried out using a specific painkiller. If necessary, bandages are made. They are usually bandaged under anesthesia.
Prognosis for Lyell’s syndrome
Usually, the prognosis of the disease can be determined by its course. In this regard, there are only three variants of the course of Lyell’s syndrome: a lightning-fast course with a subsequent fatal outcome, an acute course with a possible but optional fatal outcome (in the case of an infectious process) and a favorable course. The sooner the treatment of Lyell’s syndrome begins, the better the prognosis of the disease will be.
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