Lyell’s syndrome is a serious disease accompanied by bullous lesions of the skin and mucous membranes with detachment of the epidermis or layer of epithelial cells, often due to the use of drugs. First described by Lyell in 1956. The frequency is 0.3% of all cases of drug allergy. The etiology in the bulk of cases is unknown, often (in 30-50%) associated with the use of drugs (sulfonamides, antibiotics, NSAIDs, etc.). The pathogenesis is unclear. In cases associated with taking drugs, it is suggested that allergic mechanisms are involved (types III and IV of allergic reactions), where the product probably plays the role of a hapten that is fixed to skin cell proteins. Some researchers consider Lyell’s syndrome as the most severe manifestation of erythema multiforme.
- Acute onset with a rise in temperature up to 39-40 ° C
- The appearance of erythematous spots on the skin and mucous membranes (erythematous stage), within 2-3 days turning into flabby thin-walled blisters of irregular shape (bullous stage) with a tendency to merge, easily torn with erosion of extensive surfaces (desquamation stage). the surface resembles a burn with boiling water II-III degrees
- Positive symptoms of Nikolsky (epidermal detachment) and Asbo-Hansen (when pressing on the bladder, its size increases due to detachment of the epidermis along the periphery of the bladder under the influence of increased pressure of its contents)
- The defeat of the oral mucosa, starting with aphthous and developing to necrotic-ulcerative stomatitis
- Damage to the genital organs (vaginitis, balanoposthitis)
- Hemorrhagic (with the transition to ulcerative necrotic) conjunctivitis is the earliest manifestation of the disease
- Severe general symptoms with increasing intoxication caused by loss of fluid and protein through the affected eroded planes, impaired water-salt balance, development of infectious lesions (often pneumonia, secondary infection of the skin), bleeding from the gastrointestinal tract up to death.
Tactics of conducting
- Patients are subject to mandatory hospitalization in the intensive care unit or intensive care unit
- Patients are treated as burn patients (a burn tent is desirable) under the most sterile conditions to prevent exogenous infection.
- Applied drugs are subject to immediate cancellation. Drug therapy
- Local treatment
- The affected areas of the skin are treated with solcoseryl jelly or cream, probably the use of red
Ski Kastemany. Blisters are not recommended.
- Thorough oral hygiene: rinsing with hydrogen peroxide, 10% soda solution
- Treatment of the affected mucous membranes of the genital organs
- Care of the mucous membrane of the eyes: laying eye hydrocortisone ointment 3-4 r / day.
- Systemic therapy
- Glucocorticoids: preferably IV methylprednisolone 0.25–0.5 g/day to 1 g/day in most severe cases for the first 5–7 days, followed by dose reduction
- Detoxification and rehydration therapy
- Antibacterial therapy in case of secondary infection.
The prognosis is unfavorable for hyperacute course, late onset of active therapeutic measures, as well as the addition of a secondary infection. Lethality can reach 30-60%. Complications
- Blindness due to deep lesions of the conjunctiva
- Conjunctivitis and photophobia for several months.
Synonym. Toxic epidermal necrolysis also Erythema multiforme exudative ICD. LSI.2 Toxic epidermal necrolysis [Lyell’s syndrome]