Lyell's syndrome( epidermal toxic necrolysis, allergy to antibiotics) - Causes, symptoms and treatment. MF.
Lyell's syndrome is a toxic and allergic skin and mucous membrane damage, often accompanied by changes in the internal organs and nervous system. Synonyms: LyeU-syndrom, epidermal acute necrolysis, epidermal toxic necrolysis, toxic acute epidermolysis, skin-allergic epidermal necrolysis, necrotizing vascular allergic with predominant skin and mucous membrane damage.
Lyell syndrome is a serious disease, accompanied by a bullous lesion of the skin and mucous membranes with an epidermal layer detachment or epithelial cell layer, often caused by the use of drugs. Lyell's syndrome is a serious, immunoallergic, drug-induced disease that threatens the patient's life, which is an acute cutaneous visceral pathology and is characterized by intense epidermal detachment and necrosis of the epidermis with the formation of extensive blisters and erosions on the skin and mucous membranes.
But in general, there are still no clear definitions of Lyell and Stevens-Johnson syndromes. Both syndromes are among the most severe forms of drug toxemia. Some people consider Lyell's syndrome to be the most severe variant of Stevens-Johnson syndrome, however, according to other researchers, pathogenetically these are different diseases and Lyell's syndrome is a toxic manifestation of idiosyncrasy from epithelial cells to certain drugs or their metabolites.
Causes of Lyell's Syndrome
All causes of Lyell's syndrome are not fully understood. Lyell's syndrome occurs as a result of taking some medicines( LS: sulfonamides, antibiotics, barbiturates), causing to necrosis of all layers of the skin .Therefore, it is important to remember that self-medication, especially with the use of such strong means as antimicrobial, is dangerous for health.
The pathogenesis of Lyell's is unclear. In cases associated with taking drugs, involve allergic mechanisms( III and IV types of allergic reactions), where the drug probably plays the role of a hapten that fixes to the proteins of skin cells. Some researchers consider Lyell's syndrome as the most severe manifestation of erythema multiforme.
The appearance of erythematous spots on the skin and mucous membranes( erythematous stage), which for 2-3 days turn into flabby thin-walled bubbles of irregular shape( bullous stage) with a tendency to fuse, easily tearing with erosion of extensive surfaces( desquamation stage).In the midst of the disease, the affected surface resembles a burn with boiling water of the II-III degree
Positive symptoms of Nikolsky( an eruption of the epidermis) and Asbo-Hansen( with pressure on the bladder, its size increases due to a detachment of the epidermis around the periphery of the bladder due to the increased pressure of its contents)
Lesion of the mucous membrane of the cavitymouth, starting with aphthous and developing to necrotic ulcerative stomatitis
Lesion of genitals( vaginitis, balanoposthitis)
Hemorrhagic( with transition to an ulcernecrotic) conjunctivitis is the earliest manifestation of the disease • A pronounced general symptomatology with increasing intoxication caused by the loss of fluid and protein through the affected eroded surfaces, violation of the water-salt balance, the development of infectious lesions( often pneumonia, secondary skin infection), bleeding from the gastrointestinal tractbefore the fatal outcome.
Differential diagnosis of is performed with a staphylococcal syndrome of "burned" skin. Diagnosis of acute epidermal necrolysis is established on the basis of anamnesis( taking medications), a clinical picture( acute development of the disease, severe general condition of the patient, blisters against the erythema, symptom of detachment of the epidermis, damage to the mucous membranes of the mouth, eyes, genitals), laboratory data, pathomorphologicalstudies( necrolysis of the epidermis, the formation of intra- and subepidermal blisters).
The Lyell syndrome should be distinguished from the scalded skin syndrome. Lyell's syndrome occurs mainly in adults, usually caused by drugs and is accompanied by a higher mortality. For differential diagnosis, a puncture biopsy of the skin is carried out with the study of frozen sections: in the case of scalded skin, the epidermal stratum corneum exfoliates; in Lyell's syndrome, the entire epidermis is completely removed.
Symptoms of Lyell's Syndrome
The disease begins with a high body temperature up to 39-40 ° C, a sharp weakness, sometimes a sore throat. Bubbles appear on the skin and mucous membranes. With the appearance of rashes the patient's condition deteriorates sharply. After opening the blisters on the skin, a large, wet, wound surface remains. The disease can be accompanied by the defeat of internal organs - liver, kidneys, heart.
The prognosis of is unfavorable in case of overcurrent, late onset of active therapeutic measures, and secondary infection. The lethality can reach 30-60%.
Complications: Blindness with deep affection of the conjunctiva. Conjunctivitis and photophobia for several months. Portal about health www.7gy.ru
Treatment of Lyell's syndrome
When symptoms of Lyell's syndrome appear, an urgent hospitalization of the patient is required. Assign funds for the elimination of poisons( detoxifying), glucocorticoids, antiallergic agents.
Tactics of management
- Patients are subject to mandatory admission to the intensive care unit or intensive care unit
- Patients are burned( "burnt tent" is desirable) in the most sterile conditions to prevent exogenous infection of
- Used before the development of the drug syndrome are subject to immediate cancellation.
Drug Therapy
Local Treatment
Bubbling is not recommended. Irrigation of erosions with glucocorticoid aerosols( oxytetracycline + hydrocortisone, hydroxy cyclisol, etc.).Lubrication of erosion with aqueous solutions of analyte dyes;on wetting erosions appoint lotions with disinfectants( 1-2% of the boric acid solution, r. Castellani).Apply creams, oil mash, xeroform, solcoserilovuyu ointments, ointments with HA( betamethasone + salicylic acid, methylprednisolone aceponate).When the mucous membrane of the oral cavity is affected, astringent, disinfecting pests are shown: chamomile infusion, boric acid solution, borax, potassium permanganate for rinsing. Also used are aqueous solutions of aniline dyes, rr-borax in glycyrin, egg white. If the eyes are affected, zinc or hydrocortisone drops are used.
Systemic therapy
GK: preferably iv methylprednisolone from 0.25-0.5 g / day to 1 g / day in the most severe cases during the first 5-7 days with subsequent dose reduction. Detoxification and rehydration therapy. In order to maintain the water, electrolyte and protein balance - infusion up to 2 liters of fluid per day: rheopolyglucin or hemodez, plasma and / or albumin, isotonic sodium chloride solution, 10% calcium chloride solution, Ringer's solution. With hypokalemia, protease inhibitors( aprotinin) are used. The systemic use of antibiotics is indicated in secondary infection under the control of microflora sensitivity.
Prevention of Lyell's syndrome
Prescribing of medicines given their tolerability in the past, refusal to use medicinal cocktails. It is recommended immediate hospitalization of patients with toxemia, accompanied by common phenomena, fever, and treatment with glucocorticoids in large doses. Persons who have undergone Lyell's syndrome, within 1-2 years, should be limited to the provision of preventive vaccinations, stay in the sun, the application of hardening procedures.