Lyell's syndrome (toxic epidermal necrolysis, acute epidermal necrolysis) - a heavy toxic - allergic mucous membranes and skin, which is often accompanied by changes in the nervous system and internal organs.The disease is characterized by intense necrosis and exfoliation, with the formation of large blisters and erosions on the mucous membranes and skin
Causes of Lyell's syndrome
Lyell's syndrome is the most severe manifestation of drug allergy.Its origin is in most cases associated with the reception of various drugs.The leading role in the development of the disease is removed sulfanilamides (about 45%);followed by antibiotics (40%) - erythromycin, tetracyclines, penicillins, etc .;anticonvulsants (10%) - phenobarbital, carbamazepine, fentoin etc .;anti-inflammatory drugs (from 5 to 10%) - analgesic, aminopyrine, salicylates, phenylbutazone;anti-TB drugs (1.3%) - isoniazid.In addition, the emergence of Lyell's syndrome can trigger the reception and other groups of drug
Lyell Syndrome Symptoms
disease develops very acutely with improved overall body temperature to 39-40 ̊S, headaches and joint pain, sore throat, appearance of soreness and burning of the skin.On the skin of the trunk, limbs, face, genitals, as well as to the oral mucosa appears polymorphic, blotchy, erythematous, painful on palpation rash, prone to mergers.Over time the rash takes bluish - gray color.After the mechanical action on the data centers of the rash, the epidermis is separated solid formation.On-site formed erythematous spots appear flabby large bubbles filled with serous - bloody or serous contents, which after opening the form rather extensive erosive surface, surrounded by areas of edema and congested skin.These erosive changes can occur on almost the entire surface of the limbs and trunk, as well as to develop on the mucous membranes of the genitals, lips and mouth.Due to the large loss of fluid from the erosive surface, there is a significant dehydration.Over time, these surfaces develop erosive ulcer formation.
During the progression of Lyell's syndrome decreased blood pressure, increased erythrocyte sedimentation rate, there is tachycardia, a positive sign of Nikolsky, present expressed strong manifestations of intoxication.In the case of a favorable course of the disease, the reverse development occurs in most cases the end of the fourth week from the onset of the pathological process.
If Lyell's syndrome in children is caused by severe, it is joined by a secondary infection, there are inflammatory changes in the airways , nephritis, myocarditis, develops dehydration.As a result of cardio - vascular insufficiency and anuria possible death
Lyell Syndrome Lyell's syndrome diagnosis is based on clinical presentation, history and laboratory data.To establish the role of a drug substance or in the development of the process are carried out immunological tests (reaction of blasttransformation etc.).Also mandatory is necessary to conduct a differential diagnosis of pemphigus, erythema multiforme and other types of drug reaction
Treatment Lyell's syndrome
When typical for Lyell's syndrome symptoms need immediate hospitalization in the intensive care unit or the intensive care unit.Patients with the disease are as a burn, with a special "burn the tents" (to create the most sterile conditions and prevent exogenous infection).Without exception, all drugs used to develop this syndrome, are subject to immediate cancellation.Oral and parenteral glucocorticosteroids administered.Extensive erosive surface treated by open way by treating them with vitamin A and vegetable oils.To make up for the loss of fluid and to remove intoxication carried colloidal infusion therapy and brine.When you join secondary infection is possible the use of antibiotics and antiseptics.Suffice it worked well in the treatment of Lyell's syndrome plasmapheresis and immunomodulating agents.
forecast future life in cases of pulmonary clinical forms of Lyell's syndrome and adequate therapeutic tactics is quite favorable.Children with severe course of the disease there is a serious threat to life.
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