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Toxic epidermal necrolysis (TEN) is the most severe of the Stevens-Johnson syndrome-toxic epidermal necrolysis (SJS-TEN) spectrum. It is characterized by epidermal exfoliation and mucositis and carries an average mortality of 25 per cent. In this 6-year retrospective study, we reviewed 23 patients with drug-induced SJS-TEN. The most common causative agents were anticonvulsants and traditional medication. The mean time of onset of symptoms was 4.1 days (range 1-16 days) and the mean body surface area involved was 57.2 per cent (range 30-90 per cent). The condition was most commonly associated with ocular complications and sepsis. Using our treatment protocol in a burns centre, we were able to achieve a mean time to complete healing of 20.2 days (range 7-53 days) and a mean duration of hospitalization of 34.1 days (range 7-134 days). The length of hospital stay was prolonged when non-ocular complications supervened. The percentage mortality in our series was 10 per cent. It is our contention that the best results are obtained with treatment of the SJS-TEN patient in a burns centre with an internist, dermatologist and infectious disease specialist as part of the management team.
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PMID:Toxic epidermal necrolysis in a burns centre: a 6-year review. 878 18

A total of 182 critical patients with sepsis (n = 62), prolonged compression syndrome (PCS) (n = 41), toxic allergic dermatitis (TAD) presenting as Lyell's and Stevens-Johnson's syndromes (n = 54), and stages I-II status asthmaticus (SA) (n = 25) were examined. Statistical computer processing of the data by the method of comparing the means and analysis of correlations revealed a cascade of numerous mediators and metabolites which can be regarded as manifestations of the systemic inflammatory response syndrome (SIRS). This syndrome develops as a reaction to infection, toxic products of microorganisms, ischemic reperfusion lesions, and humoral cytotoxic reactions and is responsible for the formation of multiple organ disorders. Inefficiency of 7-10-day traditional intensive care necessitated active repair of the natural defense mechanisms and addition to the therapeutic complexes of hemo- and plasmaxenosplenoperfusion variants developed by the authors. Despite multiple organ disorders, splenoperfusion decreased the period of critical state from 24 to 12 days in sepsis, from 28 to 18 days in TAD, from 14 to 10 days in PCS, and from 17 to 7 days in SA. The mortality in sepsis dropped from 32.4 to 8%, in TAD from 10.7 to 4%, in PCS from 38.8 to 14.7%, and in SA from 15.9 to 5%. No additional drug therapy or equipment was needed.
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PMID:[Variants of the treatment of critical states with consideration of the pathogenesis of the systemic inflammatory response syndrome]. 951 Dec 48

Toxic epidermal necrolysis syndrome is an uncommon, acute, life-threatening disorder that involves sloughing of skin at the dermal-epidermal junction with associated mucositis. Between 1985 and 1995, 36 patients were treated for toxic epidermal necrolysis syndrome, at the Baltimore Regional Burn Center. A retrospective chart analysis was performed to discover significant determinants of mortality. Ninety-seven percent of the patients (35 of 36) were referred from outside institutions after an average of 6.3 +/- 0.8 days. Analysis of the data shows that patients who survived had been referred 7.5 days earlier than nonsurvivors (4.0 +/- 0.5 days versus 11.5 +/- 1.4 days, p < 0.001). When the patients were separated into two groups on the basis of time of referral, those referred "early" (< or = 7 days) had a mortality rate of 4 percent (1 of 24) versus 83 percent (10 of 12) for those referred "late" (> 7 days) (p < 0.001). Data were available from transferring institutions for 21 of the 36 patients. Analysis of the microbiologic data from these 21 patients revealed bacteremia, and subsequent death occurred in 100 percent (6 of 6) of the patients referred with positive cultures, whereas bacteremia developed in only 33 percent (5 of 15) of the patients referred with negative cultures, for a mortality rate of 7 percent (1 of 15). In addition, 86 percent (6 of 7) of the patients who were referred late (> 7 days) had positive cultures on referral. The current trend toward prolonged treatment in outside facilities before referral to a burn center is detrimental to the care of patients with toxic epidermal necrolysis syndrome. The overall rate of bacteremia, septicemia, and mortality is significantly reduced with early (< or = 7 days) referral to a regional burn center.
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PMID:Toxic epidermal necrolysis syndrome: mortality rate reduced with early referral to regional burn center. 973 18

Toxic epidermal necrolysis (TEN) is an exfoliative skin disorder that may involve a large body surface area and mucosal surfaces. The microscopic changes that occur with this condition are similar to those that occur with superficial dermal burns, such as dermal detachment from the underlying dermis. Complications of TEN are related to the loss of the epithelial skin barrier and include pain, fluid and electrolyte loss, and an increased risk of sepsis. The treatment of a patient with TEN is best accomplished in a burn unit, where expert treatment of these complications can be provided. Medical treatment includes the administration of immunosuppressive therapy and the discontinuation of any previous corticosteroid treatment. Surgical management includes the debridement of necrotic areas. In this article, the surgical management of 8 consecutive patients with TEN who were admitted to the intensive care burn unit at the Hospital Universitario de Getafe in Madrid, Spain, from 1996 to 1998 is described. These patients were treated with extensive early debridement of necrotic skin areas followed by wound coverage with Biobrane (Dow B. Hickam, Inc, Sugarland, Tex), a temporary semisynthetic skin substitute. Skin coverage with this material decreases pain and fluid loss, and it possibly facilitates epithelization and decreases the risk of sepsis, without adverse side effects. This semisynthetic material meets some standards of an ideal skin substitute: it is easy to use, provides several beneficial physiologic effects, and improves patients' comfort. In the 8 cases of patients with TEN that were studied, the use of Biobrane skin substitute for the coverage of massive areas of detached skin was found to be an important aspect of treatment.
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PMID:Skin coverage with Biobrane biomaterial for the treatment of patients with toxic epidermal necrolysis. 1050 29

Toxic epidermal necrolysis is a severe, life-threatening illness with up to one-third mortality. We report a retrospective analysis of all cases treated in Royal Perth Hospital over a 20-year period from July 1978 to June 1998, by analysis of medical records. A total of 12 patients with an age range of 23-73 years was identified. The female to male ratio was 2:1, with age of onset earlier in females. All cases were associated with medications, most commonly antibiotics, anticonvulsants and allopurinol. The mortality rate was one-third (four deaths), mostly resulting from cardiorespiratory failure, renal failure and sepsis. Risk factors for death were advanced age and severe underlying disease, including diabetes, alcoholic liver disease sepsis and malignancy. Among the six patients treated with systemic corticosteroids there was only one death. Treatment with corticosteroids appeared to be beneficial, with such patients having both fewer complications and a lower mortality rate.
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PMID:Toxic epidermal necrolysis in Western Australia. 1071 97

Toxic epidermal necrolysis is a rare but acute life-threatening syndrome in which the epidermis blisters and peels in large sheets. In general, patients with this syndrome are managed as severe second-degree burn patients, but special consideration should be given to mucous membrane involvement that reduces fluid intake and worsens the fluid deficit, systemic involvement that makes these patients hemodynamically unstable, and progression of cutaneous lesions that enhances the risk of infection and sepsis.
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PMID:Anesthetic management of toxic epidermal necrolysis: report of three adult cases. 1133 Nov 76

Severe skin adverse drug reactions can result in death. Toxic epidermal necrolysis (TEN) has the highest mortality (30-35%); Stevens-Johnson syndrome and transitional forms correspond to the same syndrome, but with less extensive skin detachment and a lower mortality (5-15%). Hypersensitivity syndrome, sometimes called Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS), has a mortality rate evaluated at about 10%. Drug reactions are self-limited diseases and therefore, generally treatment is symptomatic. Prompt diagnosis, identification of, and early withdrawal of all suspect drugs are the most important preliminaries. The management of the patients must be undertaken in specialized intensive care units, with the same main types of therapy as for burns: warming of the environment, correction of electrolyte disturbances, administration of a high caloric enteral intake, and prevention of sepsis. Efficacy of drugs used in some case reports is difficult to evaluate: intravenous immunoglobulins, cyclosporin, cyclophosphamide, pentoxyfilline, and thalidomide have all been tried. Corticosteroid use is debated and is probably deleterious in late forms of TEN. For DRESS, corticoids are used in cases of life-threatening systemic impairment. Specific nursing care and adequate topical management reduce associated morbidity and allow a more rapid re-epithelialization of skin lesions. After healing, follow-up is needed for ophthalmologic and mucous membrane sequelae. Sunblocks are recommended. Testing for glycemia must be done. Avoidance of the responsible drug and chemically related compounds is essential for the patient and first-degree relatives.
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PMID:Treatment of severe drug reactions: Stevens-Johnson syndrome, toxic epidermal necrolysis and hypersensitivity syndrome. 1216 15

Toxic epidermal necrolysis syndrome is an uncommon, acute, life-threatening, medication-induced disorder with a reported mortality rate of 20 to 60 percent. Different variables have been identified as risk factors. The extent to which these variables, when combined, affect the mortality and outcome in toxic epidermal necrolysis syndrome patients has not yet been reliably defined. Because of the high mortality rate, the logistic analysis of studied variables was performed to see whether a prognostic algorithm could be developed to aid the management of these patients. Thus, a retrospective review of 56 consecutive toxic epidermal necrolysis syndrome patients treated over a period of 13 years was undertaken in the authors' burn center. The demographics included age, sex, race, and total body surface area involved. The other variables studied were comorbidities, sepsis, steroid administration, and the interval between onset of rash and burn center admission. Data were subjected to Fisher's exact test and logistic analysis. Thirty-six patients (64.3 percent) were alive and 20 (35.7 percent) died. Univariate analysis indicated that the male/female ratio was 12:24 for survivors and 9:11 for nonsurvivors (p = 0.4). The white/nonwhite ratio was 80 percent for survivors and 54 percent for nonsurvivors (p = 0.58). The median age was 48.4 +/- 22.8 years (survivors, 41.7 +/- 22.0; nonsurvivors, 60.5 +/- 19.5; p = 0.002). Total body surface area involvement for survivors was 56.9 +/- 32 and 77.7 +/- 21 for nonsurvivors (p = 0.005). The presence of one or more comorbidities between the two groups differed (53 percent survivors and 90 percent nonsurvivors, p = 0.007), indicating eight times higher odds of dying in their presence. The average time between the onset of symptoms and admission to the burn unit was 5.25 +/- 3.4 days for survivors and 7.15 +/- 4.5 days for nonsurvivors (p = 0.08). The presence of sepsis (19.4 percent survivors, 95 percent nonsurvivors, p < 0.001) decreased odds for survival by a factor of 79. Steroids given as a single dose or multiple doses before the patient's transfer to the burn unit were not significantly associated with death (44 percent survivors, 65 percent nonsurvivors, p = 0.14). A multivariate logistic regression model yielded odds ratios of 1.11 (95 percent confidence interval, 1.03 to 1.19) for age in years, 304 (95 percent confidence interval, 8.83 to 10,400) for the presence of sepsis, and 1.03 (95 percent confidence interval, 0.99 to 1.08) for body surface area in percent. All those entering the burn unit with sepsis died. Equivalently, no survivors had sepsis before admission to the burn unit, whereas 55 percent of nonsurvivors had sepsis before admission and 40 percent developed sepsis after admission. When investigating the effect of age and sepsis, no patients over age 60 ever having sepsis survived, whereas all those who were under 60 and without sepsis survived. Likewise, all patients whose age was over 60 and whose total body surface area involved was over 60 percent died. The main factors contributing to the mortality from toxic epidermal necrolysis syndrome, when considering covariates separately, are the presence of sepsis at any time (odds ratio, 79), the presence of comorbidities (odds ratio, 8.05), age, and total body surface area, whereas multivariate models suggested age (odds ratio per year of additional age, 1.11), total body surface area (odds ratio per additional percent of body surface area, 1.03), and the presence of sepsis (odds ratio, 304). By using the actual coefficients in the logistic model, the log odds that the patient will die as the result of his or her condition can be summarized in the following formula: -11.5 + (10 percent of the patient's age + 3 percent of total body surface area + 5.75 if sepsis is present). The awareness of the importance of these covariates, and their early recognition as risk factors, should offer a focused approach to the patients' management and improve their outcome.
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PMID:Outcome of patients with toxic epidermal necrolysis syndrome revisited. 1217 37

Toxic epidermal necrolysis (TEN) is a disease occurring with low-incidence but has a relatively high mortality rate. Sepsis is the predominant cause for life-threatening complications in TEN but severe mucosal damage represents a further complication which may delay convalescence. We report a case of TEN in a 51-year-old man which eventually spread to include the whole skin surface. The long-term and comprehensive treatment focused on support of the organ failure as well as wound treatment. The extent of involvement of the intestinal tract, the sustained laryngeal stenosis and the pronounced saddle-nose were unusual. It appears necessary to treat TEN in facilities which offer intensive care and are able to manage extensive skin damage. Burns units offer the best conditions for its management.
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PMID:[Drug-induced toxic epidermal necrolysis with involvement of the intestinal and respiratory tract. A case report]. 1239 72

Toxic epidermal necrolysis is a rare but life-threatening exfoliating disease of the skin and mucous membranes, commonly considered a drug sensitivity reaction. In this review of the literature we discuss the epidemiology, etiology, and pathology. We show diagnostic problems and current treatment strategies. Many of the problems associated with the disease, such as wound infection, sepsis, nutritional support, and pain management, are similar to problems in severely burned patients. Burn centers provide optimal logistics and knowledge to diagnose and treat this serious disease entity.
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PMID:[Toxic epidermal necrolysis. A case for the burn intensive care unit]. 1274 94


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