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Lyell's syndrome (toxic epidermal necrolysis) in children

 
, medical expert
Last reviewed: 07.07.2025
 
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Lyell's syndrome is one of the most severe drug-induced disorders. It is rare in children.

It develops with the use of several medications (antibiotics, sulfonamides, nonsteroidal anti-inflammatory drugs, anticonvulsants), less often - with blood or plasma transfusions. Hereditary predisposition plays a certain role.

In pathogenesis - development of an allergic reaction of the Arthus phenomenon type - necrosis of all layers of the epidermis, dermis, thrombocapillaritis and thrombovasculitis. Characteristic is the Nikolsky symptom - detachment of the epidermis with minor mechanical impact.

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Symptoms of Lyell's syndrome

The onset of the disease is acute. The condition is severe. Hyperthermia. Intoxication and dehydration are expressed. Erythematous painful elements appear on the skin with a rapid transformation into large bullous blisters with subsequent formation of extensive erosive surfaces. Up to 80-90% of the entire skin surface is affected, with the exception of the scalp. Externally, the child resembles a patient with burns. Toxicosis, microcirculation disorders, hypovolemia develop, and DIC syndrome may develop. All these disorders lead to toxic-allergic lesions of the internal organs: heart, kidneys, liver. Skin infection leads to generalization of the infection and the development of hyperergic sepsis and pneumonia.

Mortality is 30-50%. With timely and adequate therapy, improvement in the condition occurs in the 2nd-3rd week, erosions heal by the end of the month from the onset of the disease and pigmentation remains in their place.

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Treatment of Lyell's syndrome

Cancel all medications that the child was receiving. The patient is hospitalized in the resuscitation or intensive care unit, and if possible, in a burn tent. Sterile linen is mandatory! It is important to ensure the "temperature protection" mode. Prescribe parenteral prednisolone at a dose of 5-10 mg / kg per day, infusion therapy, heparin at a dose of 10-15 U / kg per hour intravenously by drip, broad-spectrum antibiotics. In very severe cases, plasmapheresis is indicated in combination with pulse therapy with prednisolone.

Local treatment of Lyell's syndrome is similar to treatment of burns.

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