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It hurts your head, throat or stomach – when passing a kiosk or passing a gas station, we buy an over-the-counter painkiller and the vast majority of us feel relieved after taking it. But what if toxic epidermal necrolysis occurs? The occurrence of Lyell’s syndrome cannot be predicted while taking a given preparation, therefore this disease should be a warning against unjustified use of drugs, which may even result in death.
Lyell’s syndrome, also known as toxic epidermal necrolysis, is a life-threatening skin disease most often caused by medication. It is associated with a rapidly progressive loss of epidermis over a large area of the body. As a result, the skin ceases to perform its functions – it does not protect the body against pathogenic microorganisms and against the loss of body fluids and excessive heat loss. In the absence of this natural protective barrier, infections and dehydration can easily occur.
Toxic epidermal necrolysis was first described in 1958 by Allan Lyell. It is a relatively rare disease. It is estimated that about 40 people are diagnosed annually in Poland. It affects men slightly more than women. Its occurrence in 95% of cases is caused by taking medications. More than 200 drugs have been described that can cause this disease. These are most often preparations used in the treatment of bacterial infections (mainly sulfonamides, antibiotics from the cephalosporin group and fluoroquinolones), non-steroidal anti-inflammatory drugs (especially from the oxicam group), drugs used in the treatment of epilepsy (carbamazepine, phenytoin, valproic acid, phenobarbital, lamotrigine), gout (allopurinol) and in the treatment of HIV infection (nevirapine, abacavir). Much less frequently the cause of toxic epidermal necrolysis is infection (incl. Mycoplasma pneumoniae, herpes viruses, hepatitis A virus), as well as organ transplantation.
The pathomechanism of Lyell’s syndrome has not been fully understood. It is known that in the course of this disease, apoptosis occurs – the suicidal death of epidermal cells, which leads to its necrosis, blisters on the skin and a flap separation of the epidermis from the dermis.
The initial symptoms of toxic epidermal necrolysis usually appear within the first 3 weeks of taking the drug. The disease picture then resembles a viral infection with high fever, cough, muscle pain and general weakness. Skin changes appear soon. They are painful, mainly located on the trunk and face, and cover more than 30% of the body surface. Initially, they are scattered, small, erythematous, irregular in shape, flat spots, which merge into larger spots within a few hours or days. Then, at the site of the erythematous spots, flaccid blisters, often filled with clear serous fluid, form. Apart from blisters, the epidermis covering the changed skin becomes shifting and over large areas it separates from the dermis under the influence of slight pressure. At the site of its loss, red and painful skin remains, which is easily susceptible to bacterial and fungal infections. After the lesions have healed, the skin is usually discolored and scarred.
The lesions in Lyell’s syndrome also affect the mucous membranes. In the mouth and throat they show burning sensation and pain, making it difficult, sometimes even impossible, to swallow. There, too, blisters form, which, when ruptured, leave aphthous ulcerations and bleeding, painful erosions. Minor bleeding can occur throughout the digestive tract. Similar changes may also affect the mucosa of the respiratory tract, leading to the retention of secretions in the bronchial tree, which in turn predisposes to bacterial pneumonia. Lyell’s syndrome may develop pulmonary edema and respiratory failure. When the organ of vision is affected by the disease, the eyelids are swollen and the conjunctiva is reddened with photophobia. Corneal ulcers and secondary bacterial infections may develop, such as: the presence of purulent discharge in the conjunctival sac. After eye lesions have healed, complications such as dry eye syndrome, persistent photosensitivity, eyelid adhesions, as well as corneal scarring and even blindness sometimes remain. Also, painful ulcers can form on the genitals, and erosions in the urinary tract. During the healing of these lesions, sometimes the urinary tract is narrowed. A common and dangerous complication of Lyell’s syndrome is acute renal failure.
There are no tests to find out which drugs can cause Lyell’s syndrome in a person. Additional tests are not needed to diagnose the disease itself. Its course and image are very characteristic and allow for a proper diagnosis. Laboratory tests are helpful in determining the impact of the presence of skin lesions on body functions and in planning treatment. They allow the assessment of electrolyte and protein loss and kidney function. In the event of infection, effective antibiotics can be used thanks to bacteriological tests. Histopathological examination of a section of the affected skin is usually not necessary. In case of diagnostic doubts, it allows to establish a diagnosis by excluding other diseases similar in its picture to Lyell’s syndrome, such as, for example, staphylococcal exfoliative dermatitis.
Treatment of Lyell’s syndrome is analogous to the treatment of burns. The best place to run it is the burn ward. In order to minimize the risk of contamination of epidermal skin, patients with Lyell’s syndrome should be kept in isolation. The first step in the management of patients with toxic epidermal necrolysis is to stop using the drug that is suspected of causing the disease. Topical treatment involves the use of sterile dressings that are changed daily, often with the addition of silver, which is itself antibacterial. Replenish fluids and electrolytes lost through the skin. An important element of the therapy is the use of painkillers and antipyretics. In the event of an infection, antibiotics and, often, antifungal medications should be used. The patient loses protein along with the serum fluid, so nutritional treatment with a high-calorie, high-protein diet plays an important role in the treatment of Lyell’s disease. The eyes require special attention. Artificial tears, antibiotics and steroids are used to reduce the inflammatory reaction of the organ of vision and the risk of permanent consequences such as corneal scarring and blindness. There are also attempts to treat the toxic epidermal necrolysis syndrome with plasmapheresis (plasma exchange) as well as immunosuppressive drugs (lowering immunity), but the previous studies have not clearly proven their effectiveness.
The mortality rate in Lyell’s syndrome is high, around 40%. It is mainly caused by severe infections and excessive loss of fluid through damaged skin. The risk depends largely on the extent of the skin lesions and the age of the patient at the time of the onset of the disease. For this reason, it is extremely important to prevent this dangerous disease. Patients who have undergone toxic epidermal necrolysis must absolutely avoid the drug that was responsible for the disease. However, all those who plan to use a drug should consider whether it is necessary, being aware that drug abuse carries an increased risk of serious side effects, including Lyell’s syndrome.
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