Encyclopedia

Lyell’s syndrome

Lyle’s syndrome is a severe disease characterized by the formation of emphysema lesions on the skin and mucous membranes, whereby the epidermis or layer of epithelial cells is separated, often due to the use of medicinal preparations.

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This is one of the most serious diseases in general, which is drug-induced and has an immuno-allergic origin.

To date, precise medical definitions of Lyell and Stevens-Johnson syndromes have not been established. Both diseases refer to the most severe forms of medicinal toxidermia.

Some scientists believe that Lyell’s syndrome is the most severe clinical variant of Stevens-Johnson syndrome, but according to other researchers, pathogenetically, these are different diseases and Lyell’s syndrome is a toxic manifestation of idiosyncrasy, that is, a hypersensitivity reaction , of the epithelial cells to certain drugs or their metabolites.

What are the causes?

The causes of this syndrome are not fully understood. The disease occurs as a result of taking certain medications such as sulfanilamides, antibiotics, barbiturates and leads to necrosis of all skin layers.

This is why it is important to remember that self-medication, especially when using such strong preparations as antibiotics, is dangerous to health.

The pathogenesis of Lyell’s syndrome is not clear.

In cases related to the intake of medicinal preparations, the involvement of allergic mechanisms is assumed, where the preparation probably fulfills the function of a hapten attaching to the proteins of the skin cells.

Some scientists consider this disease as the most severe manifestation of erythema multiforme.

The appeared erythematous spots on the skin and mucous membranes /erythematous stage/ after 2-3 days turn into small thin-walled blisters of irregular shape /bullous stage/ with a tendency to coalesce, easily ulcerate and spread over large skin surfaces /desquamation stage/.

At the peak of the disease, the affected skin surface looks like a burn with boiling water 2nd 3rd degree.
Positive symptoms of Nikolsky /detachment of the epidermis / and Asbo-Gansen /when pressing on the blister, its size increases as a result of detachment of the epidermis along its periphery under the action of the increased pressure of its contents/.

The mucous membranes of the oral cavity are affected, which manifests as aphthous and progresses to necrotic-ulcerative stomatitis.
The earliest manifestation of the disease is hemorrhagic conjunctivitis with a transition to ulcerative-necrotic.

Pronounced generalized symptomatology with increasing intoxication caused by the loss of fluids and proteins from the affected eroded surfaces.

Also, the water-salt balance in the patient’s body is disturbed, and secondary infectious diseases develop – most often pneumonia, secondary infection of the skin, hemorrhages in the gastrointestinal tract with an increased risk of death.

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Leil’s syndrome should be distinguished from burnt skin syndrome, as the former occurs mainly in adults, is usually drug-induced, and the risk of mortality is very high.

What are the symptoms?

The disease begins with an increase in body temperature to 39-40 degrees Celsius, those affected suddenly begin to feel weakness and sometimes a sore throat.

Blisters form on the skin and mucous membranes. With the appearance of the rash, the patient’s condition worsens sharply. The disease can be accompanied by affecting the internal organs.

Treatment of Lyle’s syndrome

When the symptoms of the disease appear, urgent hospitalization of the patient is mandatory. Medicinal preparations are prescribed to remove harmful substances from the body /detoxifiers/, glucocorticoids and anti-allergic agents.

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