Lyell ‘s syndrome is a life-threatening acute skin disease that is associated with extensive epidermolysis (detachment of the epidermis) and is due to pronounced drug intolerance or infection with staphylococci. With an incidence of about 1:1,000,000, Lyell’s syndrome is a rare disease.
What is Lyell Syndrome?
Lyell’s syndrome ( also “scalded skin syndrome”) is a rare, life-threatening acute dermatosis ( skin disease ) that is accompanied by epidermolysis (bubbly epidermal detachment) as a result of generalized subepidermal blistering. See AbbreviationFinder for abbreviations related to Lyell Syndrome.
In Lyell’s syndrome, depending on the underlying cause, a distinction is made between the drug-induced variant (toxic epidermal necrolysis or TEN), which primarily affects adults, and the staphylogenic variant (staphylococcal scalded skin syndrome), which is observed primarily in infants and young children. differentiated.
Lyell’s syndrome initially manifests itself as loss of appetite, inflammation of the mucous membranes (rhinitis) and malaise (prodomal stage). In the acute stage, persistent fever leads to generalized blistering erythema (Nikolski phenomenon) and extensive epidermolysis due to necrosis.
In addition, in many cases the mucous membranes (especially the oral mucosa) are affected by the necrosis. The skin erosions characteristic of Lyell’s syndrome result in a loss of fluids, which can derail the electrolyte and water balance.
Causes
Lyell ‘s syndrome is caused by a pronounced drug intolerance (drug Lyell’s syndrome) or an infection with staphylococci (staphylogenic Lyell’s syndrome).
The drug-induced variant of the disease is caused by an allergic-cytotoxic reaction to certain drugs taken. Drugs that can trigger such a reaction include hypnotics (hypnotics such as barbiturates), non-steroidal anti- inflammatory drugs and analgesics (painkillers such as pyrazolone derivatives), some antibiotics (e.g. sulfonamides such as cotrimaxol) and anticonvulsants (including antiepileptics such as carbamazepine, phenytoin, lamotrigine ). and allopurinol (gout medicine) were counted.
Staphylogenic Lyell’s syndrome, on the other hand, is caused by an exotoxin (exfoliatin) produced by Staphylococcus aureus. In many cases, the disease is preceded by a purulent skin or conjunctival infection, pharyngitis (inflammation of the throat) or otitis ( ear infection ). While adults usually show immunity due to antibodies that neutralize the exotoxin, this has not yet developed in infants and small children, so that a staphylogenic Lyell’s syndrome can manifest itself as a result of a cytotoxic reaction.
Symptoms, Ailments & Signs
Lyell syndrome typically presents with a runny nose, fever, and other flu symptoms. Accompanying this, those affected feel an increasing exhaustion. The appetite decreases as the disease progresses, which can lead to weight loss and deficiency symptoms. In general, sufferers are less resilient and withdraw from social life.
Chronic diseases often affect the mental state and cause, for example, depression or an anxiety disorder. Skin changes can also occur. The visible reddening of the skin is characteristic. These erythema eventually lead to a large-scale detachment of the epidermis.
If Lyell’s syndrome is not treated, pneumonia can develop, which is associated with serious symptoms. As the disease progresses, other symptoms can appear, the nature and severity of which depends on the patient’s state of health. If the patient is already weakened by another disease, Lyell’s syndrome can cause serious cardiovascular disease.
Possible symptoms are sweating, tachycardia, nervousness and panic attacks. In severe cases, it can lead to circulatory collapse or even heart failure. Symptoms of Lyell’s syndrome come on suddenly and worsen as the disease progresses. If the patient receives immediate medical attention, the symptoms subside after a few days or weeks.
Diagnosis & History
In addition to the clinical picture, Lyell’s syndrome is diagnosed on the basis of a skin biopsy with subsequent histological examination. The finding also serves to determine the specific variant present. For example, in the drug-induced variant, cleft formation and detachment can be detected throughout the epidermis, while in staphylogenic Lyell’s syndrome, detachment of the stratum corneum (outermost skin layer) from the stratum granulosum (granular cell layer of the skin) can be observed.
Lyell’s syndrome should be differentiated from coarse impetigo contagiosa, scarlet fever and Stevens-Johnson syndrome in the differential diagnosis. Drug-induced Lyell’s syndrome has a mortality rate of about 30 to 50 percent, although this rate can be reduced to 20 percent with consistent and adequate therapy. With early therapy and the absence of possible complications ( pneumonia, sepsis ), the prognosis of staphylogenic Lyell’s syndrome is favorable.
Complications
Lyell’s syndrome often causes those affected to suffer from fatigue and exhaustion. Loss of appetite also occurs and those affected show a significantly reduced resilience. Lyell’s syndrome also severely restricts social life, as those affected no longer actively participate in social life.
This can also lead to various psychological problems or depression. The syndrome also causes fever or a runny nose. Without treatment, Lyell’s syndrome can also lead to pneumonia, which is associated with serious symptoms and complications. The immune system of those affected is weakened by this syndrome, making it easier for inflammation and infections to occur. Contact with the outside world must also be avoided to prevent further infections.
The treatment of Lyell’s syndrome is causal and depends on the cause. In the busy cases, the complaints can be treated well, whereby there are usually no special complications. However, it is not uncommon for the affected person to be placed in an artificial coma if the pain becomes too severe due to the skin changes. Lyell’s syndrome may also lead to a reduction in the patient’s life expectancy.
When should you go to the doctor?
Flu symptoms such as a runny nose or fever indicate an illness that needs to be medically clarified. It is best for those affected to go to the dermatologist, who can diagnose or rule out Lyell’s syndrome and then initiate appropriate therapy. Accompanying symptoms such as red spots on the skin, swelling or bouts of fever indicate an advanced disease that must be treated immediately. In addition, if the person keeps falling asleep or suffers from open wounds, they must be taken to a hospital.
The relatives should keep a close eye on the sick person and call the emergency services if their state of health deteriorates. This is especially true if there are signs of sepsis or liver or kidney failure. Since Lyell’s syndrome can lead to the death of the patient if left untreated, a medical evaluation is urgently needed as soon as the patient noticeably deteriorates physically or mentally. Due to the high risk of side effects and consequential damage, close consultation with the doctor is required during and after the treatment. Depending on the symptoms, Lyell’s syndrome is treated by a dermatologist, internist or organ specialist.
Treatment & Therapy
If Lyell’s syndrome is suspected, immediate intensive care therapy and consistent monitoring of the affected person is indicated. In addition, those affected who are immunocompromised usually require reverse isolation to protect against secondary infections, through which potential transmission routes as a result of contact with the outside world are prevented.
As part of a symptomatic therapy, the same therapeutic measures are usually used as are used for large-area burns. This includes close monitoring of the laboratory parameters, infusions to compensate for the loss of fluid, electrolytes and proteins via the open skin lesions, sterile and antiseptic wound care with possible surgical sanitation of the necrotic skin areas, intensive local care and positioning of the affected person on an air cushion or water bed to prevent additional pressure-induced detachment of the skin.
If there is drug-induced Lyell’s syndrome, all drugs that may have triggered the disease and are not vital are discontinued and high-dose glucocorticoids are infused intravenously. For the prophylaxis of further infections or in the case of a superinfection that has already occurred, therapy with antibiotics, which have a low allergy potential, may also be indicated.
In the case of staphylogenic Lyell’s syndrome, antibiotic therapy with high-dose, semi-synthetic β-lactam antibiotics is the priority, while the administration of glucocorticoids is contraindicated. Due to the extremely painful skin changes, many sufferers of Lyell’s syndrome are also placed in an artificial coma.
Outlook & Forecast
If Lyell’s syndrome is treated in time and there are no complications such as blood poisoning or pneumonia, the prognosis is good. The skin complaints then subside within ten to 14 days. Scars only remain in very rare cases. If Lyell’s syndrome is detected in the early stages, an onset of the disease can sometimes be avoided. For this purpose, antibiotics are administered to the patient, which are intended to kill the pathogens. If the treatment is successful, the patient’s condition improves within a day or two.
If left untreated, Lyell’s syndrome can cause complications such as scarring and nerve damage. The optical changes are then often accompanied by chronic pain. In order to ensure a positive healing process, the patient must be examined regularly by a doctor. If necessary, the medication must be adjusted or specific symptoms treated.
A specialist doctor can give the final prognosis, taking into account other factors such as the patient’s state of health, family history and any concomitant diseases. The disease usually subsides more quickly in children than in adults, in whom the disease often persists for one to two weeks.
Prevention
As a rule, Lyell’s syndrome cannot be prevented. Drug intolerances often only become evident when the specific preparation is taken. The risk of Lyell’s syndrome can be reduced or at least the effects of the disease can be minimized through early diagnosis through the sustainable use of medication and precise self-monitoring when taking potentially triggering substances.
Aftercare
In the case of skin diseases, the aftercare measures usually depend very much on the exact severity, so that no general prediction can usually be made. Lyell syndrome needs to be evaluated and treated by a doctor in the first place to prevent further complications or symptoms.
A high standard of hygiene can also have a positive effect on the course of such diseases. In most cases, these diseases are treated by applying creams or ointments and taking medication.
The person concerned should pay attention to regular use and the right dosage in order to permanently relieve the symptoms. Regular check-ups by a doctor are very important. In most cases, Lyell’s syndrome does not negatively affect the life expectancy of the person affected.
You can do that yourself
Because Lyell’s syndrome is a life-threatening condition, medical treatment and care is essential. Self-help measures can only relate to support and care provided by relatives.
In the event of drug intolerance, the respective drug should be discontinued or replaced by another after consultation with a doctor. Possible symptoms such as fever and exhaustion can be counteracted by resting in bed and avoiding unnecessary stress. The affected person is usually dependent on an inpatient stay. Loving care by relatives or friends has a very positive effect on the course of the disease. Extensive discussions with close confidants can prevent and treat psychological problems.
If children are affected by Lyell’s syndrome, they should always be informed about the possible consequences and complications of the disease. Furthermore, in many cases, contact with other people affected by Lyell’s syndrome has a very positive effect on the disease. Here, the exchange of information and possible mutual emotional support should be emphasized. Whether this syndrome will be cured cannot generally be predicted.
