Lyell syndrome is a life-threatening acute skin disorder associated with extensive epidermolysis (detachment of the epidermis) due to marked drug intolerance or infection with staphylococci. With an incidence of approximately 1:1,000,000, Lyell syndrome is a rare condition.
What is Lyell syndrome?
Lyell’s syndrome (also known as “scalded skin syndrome”) is a rare life-threatening acute dermatosis (skin disease) associated with epidermolysis (vesicular epidermal detachment) due to generalized subepidermal blistering. In Lyell 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 seen primarily in infants and young children. Lyell’s syndrome is initially manifested by loss of appetite, mucosal inflammation (rhinitis), and malaise (prodomal stage). In the acute stage, generalized vesicular erythema (Nikolski phenomenon) and extensive epidermolysis due to necrosis develop with persistent fever. In addition, in many cases the mucous membranes (especially the oral mucosa) are affected by necrotization. The skin erosions characteristic of Lyell syndrome entail fluid loss, which can derail electrolyte and water balance.
Causes
Lyell syndrome is causally due to marked drug intolerance (drug-induced Lyell syndrome) or infection with staphylococci (staphylogenic Lyell syndrome). In this case, the drug-induced variant of the disease is caused by an allergic-cytotoxic reaction to certain ingested drugs. Drugs that may cause such a reaction include hypnotics (sleeping pills such as barbiturates), nonsteroidal anti-inflammatory and analgesics (painkillers such as pyrazolone derivatives), some antibiotics (e.g., sulfonamides such as cotrimaxol), and anticonvulsants (including antiepileptics such as carbamazepine, phenytoin, and lamotrigine) and allopurinol (gout medications). In contrast, staphylogenic Lyell syndrome 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 pharyngeal mucosa) or otitis (ear infection). While adults generally have immunity due to antibodies neutralizing the exotoxin, this immunity is not yet developed in infants and young children, and staphylogenic Lyell syndrome may manifest as a result of a cytotoxic reaction.
Symptoms, complaints, and signs
Lyell syndrome typically manifests as rhinitis, fever, and other flu symptoms. Accompanying these symptoms, affected individuals experience increasing fatigue. Appetite decreases in the course of the disease, which can lead to weight loss and deficiency symptoms. In general, sufferers are less able to cope with stress and withdraw from social life. Chronic illness often affects the mental state and causes, for example, depression or an anxiety disorder. In addition, skin changes can occur. Visible reddening of the skin is characteristic. These erythemas eventually lead to extensive detachment of the epidermis. If Lyell’s syndrome is not treated, pneumonia may occur, which is associated with serious symptoms. In the course of the disease, other symptoms may develop, the nature and severity of which depend on the patient’s state of health. If the sufferer is already weakened by another disease, Lyell’s syndrome can cause serious cardiovascular disease. Possible symptoms include sweating, rapid heartbeat, nervousness and panic attacks. In severe cases, circulatory collapse or even heart failure may occur. The symptoms of Lyell’s syndrome appear suddenly and worsen as the disease progresses. If the sufferer receives prompt medical attention, the symptoms subside after a few days to weeks.
Diagnosis and course
In addition to the clinical picture, Lyell syndrome is diagnosed on the basis of a skin biopsy followed by histologic examination. The findings also serve to determine the specific variant present.For example, in the drug-induced variant, cleavage and detachment can be demonstrated throughout the epidermis, whereas in staphylogenic Lyell’s syndrome, detachment of the stratum corneum (outermost skin layer) from the stratum granulosum (granule cell layer of the skin) can be observed. Differentially, Lyell’s syndrome should be differentiated from gross blistering impetigo contagiosa, scarlet exanthema, and Stevens-Johnson syndrome. Drug-induced Lyell’s syndrome has a mortality rate of approximately 30 to 50 percent, although this rate can be reduced to 20 percent with consistent and adequate therapy. With early therapy and absence of possible complications (pneumonia, sepsis), the prognosis of staphylogenic Lyell syndrome is favorable.
Complications
As a result of Lyell syndrome, it is not uncommon for affected individuals to suffer from fatigue and lassitude. Furthermore, a loss of appetite also occurs and affected individuals show a significantly reduced ability to work under pressure. Lyell’s syndrome also severely limits social life, as those affected no longer actively participate in social life. Various psychological complaints or depression can also develop as a result. Furthermore, the syndrome causes fever or colds. 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 infection to occur. Likewise, contact with the outside world must be avoided to prevent further infections. The treatment of Lyell’s syndrome is causal and depends on the cause. In busy cases, the symptoms can be treated well, and there are usually no particular complications. However, it is not uncommon for the affected person to be placed in an induced coma if the pain due to the skin lesions becomes too severe. It is also possible that Lyell’s syndrome may reduce the patient’s life expectancy.
When should you see a doctor?
Flu symptoms such as a cold or fever indicate an illness that needs medical attention. Affected individuals are best advised to see a dermatologist, who can diagnose or rule out Lyell’s syndrome and subsequently initiate appropriate therapy. Accompanying symptoms such as red patches on the skin, swelling or fever episodes indicate an advanced disease that must be treated immediately. In addition, if the affected person repeatedly falls asleep or suffers from open sores, he or she must be taken to a hospital. Relatives should keep a close eye on the sufferer and call the emergency services in the event of a deterioration in health. 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, medical clarification is urgently required as soon as the sufferer noticeably deteriorates physically or mentally. During and after treatment, close consultation with the physician must be maintained due to the high risk of side effects and subsequent damage. Lyell syndrome is treated by a dermatologist, internist, or organ specialist, depending on the symptoms.
Treatment and therapy
If Lyell syndrome is suspected, immediate intensive medical therapy and consistent monitoring of the affected individual is indicated. In addition, immunocompromised affected individuals generally require reverse isolation to protect against secondary infections, by which potential routes of transmission resulting from contact with the outside world are prevented. In the context of symptomatic therapy, the same therapeutic measures that are used for extensive burns are generally applied. These include close monitoring of laboratory parameters, infusions to compensate for fluid, electrolyte and protein loss via the open skin lesions, sterile and antiseptic wound care with possible surgical repair 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 a drug-induced Lyell’s syndrome is present, all drugs that may have triggered the disease and are not vital are discontinued and high-dose glucocorticoids are infused intravenously.In addition, for prophylaxis of further infections or if superinfection has already occurred, therapy with antibiotics that have a low allergy potential may be indicated. In staphylogenic Lyell syndrome, antibiotic therapy with high-dose semisynthetic β-lactam antibiotics is the mainstay, while the administration of glucocorticoids is contraindicated. Because of the extremely painful skin lesions, many Lyell syndrome sufferers are additionally placed in an induced coma.
Outlook and prognosis
If Lyell syndrome is treated in a timely manner and no complications occur, such as sepsis or pneumonia, the prognosis is good. Skin symptoms then resolve within ten to 14 days. Scars remain only in very rare cases. If Lyell’s syndrome is detected at an early stage, an outbreak of the disease can sometimes still be avoided. For this purpose, the patient is given antibiotics to kill the pathogens. If treatment is successful, the patient’s condition improves again within one to two days. In the absence of treatment, Lyell’s syndrome can cause complications such as scarring and nerve damage. The visual changes are then often accompanied by chronic pain. To ensure a positive healing process, the patient must undergo regular medical examinations. If necessary, medication must be adjusted or specific symptoms treated. The final prognosis can be given by a specialist, who will take into account other factors such as the patient’s state of health, family history and any concomitant diseases. In children, the disease usually resolves more quickly than in adults, in whom the disease often persists for one to two weeks.
Prevention
Lyell syndrome generally cannot be prevented. Drug intolerances often become evident only when the specific drug is taken. Sustainable medication management and close self-monitoring when taking potentially triggering substances can reduce the risk of Lyell syndrome or at least minimize the effects of the disease through early diagnosis.
Follow-up
The measures of a follow-up usually depend very much on the exact manifestation of skin diseases, so no general prediction can usually be made. First and foremost, Lyell’s syndrome must be examined and treated by a physician to prevent further complications or discomfort. A high standard of hygiene can also have a positive effect on the course of such diseases. In most cases, the treatment of these diseases is carried out by applying creams or ointments and taking medications. The affected person should pay attention to a regular application and also to the correct dosage in order to alleviate the complaints permanently. Regular checks by a doctor are very important. In most cases, Lyell’s syndrome does not negatively affect the life expectancy of the affected person.
What you can do yourself
Because Lyell syndrome is a life-threatening condition, medical treatment and care are inevitable. Self-help measures can only refer to care and nursing by relatives. In case of drug intolerance, the respective drug should be discontinued or replaced by another one after consulting a physician. Possible symptoms such as fever and exhaustion can be counteracted with bed rest and the avoidance of unnecessary stress. The affected person is usually dependent on an inpatient stay. In this case, 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 complaints. If children are affected by Lyell syndrome, they should always be informed about the possible consequences and complications of the disease. Furthermore, in many cases, contact with other Lyell syndrome sufferers has a very positive effect on the disease. Here the exchange of information and a possible mutual emotional support is to be emphasized. Whether this syndrome will be cured cannot be predicted in general.
