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Lyell's syndrome (toxic epidermal necrolysis) in children
Last reviewed: 23.04.2024
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Lyell's syndrome is one of the most severe lesions of the drug genesis. In children it is rare.
It develops with the use of several drugs (antibiotics, sulfonamides, non-steroidal anti-inflammatory drugs, anticonvulsants), less often - blood and plasma transfusions. A certain role is played by hereditary predisposition.
In pathogenesis - the development of an allergic reaction according to the type of the Arthus phenomenon - necrosis of all layers of the epidermis, dermis, thrombocapillary and thrombovasculitis. Characteristic of Nikolsky's symptom is the detachment of the epidermis with little mechanical effect.
[Symptoms of Lyell's Syndrome
The onset of the disease is acute. The condition is heavy. Hyperthermia. The phenomena of intoxication and dehydration are expressed. On the skin appear erythematous painful elements with rapid transformation into large bullous blisters with the subsequent formation of extensive erosive surfaces. Affects up to 80-90% of the entire surface of the skin, with the exception of the scalp. Outwardly the child resembles a patient with burns. Developing toxicosis, microcirculation disorders, hypovolemia, possible development of DIC syndrome. All these disorders lead to toxic and allergic lesions of the internal organs: the heart, kidneys, liver. Infection of the skin leads to the generalization of infection and the development of hyperergic sepsis and pneumonia.
Mortality is 30-50%. With timely and adequate therapy, improvement occurs in the state of 2-3 weeks, erosion heals by the end of the month from the onset of the disease and in their place remains pigmentation.
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Treatment of Lyell's Syndrome
Cancellation of all medicines that the child received. The patient is hospitalized in the intensive care unit or, if possible, in a burnt tent. Always sterile underwear! It is important to provide a "temperature protection" mode. Prescribe prednisolone parenterally at a dose of 5-10 mg / kg per day, infusion therapy, heparin at a dose of 10-15 units / kg per hour intravenously drip, broad-spectrum antibiotics. In very severe cases, the use of plasmapheresis in combination with pulse therapy with prednisolone is indicated.
Local treatment for Lyell's syndrome is similar to treating burns.