Burn unit management of toxic epidermal necrolysis.

Toxic epidermal necrolysis is the name given to a group of dermatologic disorders characterized by a separation of epidermis and dermis with a subsequent skin slough. The denuded areas have the appearance of a second-degree burn. The complications of infection, negative nitrogen balance, severe pain, and emotional instability are identical to those seen in the patient with major burns. There are difficulties in patient management and advantages in burn unit care. As with the major burn, care of the patient with skin loss from toxic epidermal necrolysis is extremely complex, requiring the expertise of a burn team along with that of the dermatologist.     

Toxic epidermal necrolysis syndrome--management and case report

Toxic epidermal necrolysis syndrome [known also as Lyell's syndrome] is a severe drug reaction, characterized by fever. systemic toxicity, extensive skin rash with blisters and exfoliation similar to that of major burns. It carries an average mortality rate of 25%. The data concerning most frequent triggering agents and the differential diagnosis is presented; An overview of the management protocol for these patient in the Burn Center of the Lebanese Hospital is exposed with a case presentation of a young woman who sustained a 95% body surface area [BSA] cutaneous loss after the ingestion of only 2 tablets of amoxycilline documented by the complication and evolution.     

Toxic epidermal necrolysis after phenytoin usage in a brain trauma patient

Toxic epidermal necrolysis is a drug-induced, rare, but life-threatening skin eruption. The main differential diagnoses are drug-induced erythema (hypersensitivity syndrome), acute graft-versus-host disease, staphylococcal scalded skin syndrome, and toxic shock syndrome. Because the therapy for toxic epidermal necrolysis and acute graft-versus-host disease differs largely from the others, it is necessary to make an accurate diagnosis. In addition to a detailed medical history, skin biopsy is mandatory because the skin eruptions are not always unequivocal. Discontinuation of the causing agent is crucial, and treatment in specialized intensive care units or burn units is supportive. Currently there is no specific treatment for toxic epidermal necrolysis. Advantages from corticosteroids, plasmapheresis, intravenous immunoglobulin, cyclophosphamide, cyclosporin, and N-acetylcysteine still remain to be established by controlled trials, or have failed to prove a benefit (thalidomide). The patient presented here demonstrates the difficulties in diagnosing toxic epidermal necrolysis in a critically ill patient. A short overview of the pathogenesis and the management of toxic epidermal necrolysis is provided.     

Toxic epidermal necrolysis: 10 years experience of a burns centre in Hong Kong

Toxic epidermal necrolysis syndrome is characterized by extensive skin rash with blisters and exfoliation similar to that of major burns. Current evidence suggests that toxic epidermal necrolysis syndrome is most likely due to cell-mediated immune response triggered by certain drugs. Recognition of the clinical situation which is similar to major burn has resulted in general agreement that these patients are best treated in a burns unit, where critical support and meticulous wound care can be carried out by experienced personnel. This represents a major step forward in the management of this condition. The 10 year experience of managing seven patients with severe toxic epidermal necrolysis in a tertiary burns centre in Hong Kong is presented and the controversies over classification and management of the disease are also discussed.     

Toxic epidermal necrolysis (TEN) and Stevens-Johnson syndrome (SJS): experience with high-dose intravenous immunoglobulins and topical conservative approach. A retrospective analysis

Toxic epidermal necrolysis (TEN) and Stevens-Johnson syndrome (SJS) are rare, drug-induced, severe acute exfoliative skin and mucosal disorders. Several treatments previously proposed have produced contradictory results in small series; in 1998 the use of intravenous immunoglobulins (IVIG) was introduced with excellent clinical findings. Our experience (1999-2005) using IVIG in the therapy of TEN/SJS, together with a local conservative approach, is reported and related to our previous treatments (1993-1998). The SCORTEN and the standardized mortality ratio (SMR) was used to evaluate the efficacy of our therapeutic modalities. Eight patients were treated before IVIG era and 23 patients have been treated with IVIG. There was no significant difference in SCORTEN between the two groups. Concerning the local approach, a conservative wound management in IVIG series replaced an extensive epidermal debridment and coverage with artificial skin substitutes of the pre-IVIG series. Overall mortality in patients treated before IVIG was 75% (6/8), in the IVIG group it decreased to 26% (6/23) with a cessation of further epidermal detachment after an average of 5 days (3-10 days) from the onset of the therapy. The SMR showed a trend to lower actual mortality (not significative) with IVIG treatment than the predicted mortality (SMR=0.728; 95% CI: 0.327-1.620).     

Aquacel Ag with Vaseline gauze in the management of toxic epidermal necrolysis (TEN)

Toxic epidermal necrolysis (TEN) is a rare condition with potentially high mortality and involves severe exfoliative disease of the skin and mucous membranes induced by drugs. The reported fatality of TEN varies widely from 20% to 60%. The technique for TEN wound coverage described in this article involves the use of various dressings.     

Fatal phenytoin-related toxic epidermal necrolysis: case report

Toxic epidermal necrolysis is a rare but often fatal hypersensitivity reaction to numerous agents, including most anticonvulsants. The authors present a case of fatal phenytoin-related toxic epidermal necrolysis in a patient who was given prophylactic anticonvulsant therapy after he sustained a moderately severe closed head injury. The typical course and current management of toxic epidermal necrolysis are reviewed, as are the indications for the prophylaxis of posttraumatic epilepsy.     

Improved burn center survival of patients with toxic epidermal necrolysis managed without corticosteroids.

Fifteen consecutive patients with toxic epidermal necrolysis or the Stevens-Johnson syndrome managed without corticosteroids after transfer to the burn center (group 2) are compared to a previous consecutive group of 15 who received high doses of these drugs (group 1). Group 2 had a 66% survival, which was a significant improvement compared to the 33% survival in group 1 (p = 0.057). In group 1, mortality was associated with loss of more than 50% of the body surface area skin. In group 2, mortality was related to advanced age and associated diseases. Age, extent of skin loss, progression of skin loss after burn center admission, incidence of abnormal liver function tests, and the incidence of septic complications were not significantly different in the two groups (p greater than 0.10). The incidence of detected esophageal slough was similar in both groups. Nonsteroid (group 2) management was associated with a decreased incidence of ulceration of gastrointestinal columnar epithelium, Candida sepsis, and an increased survival after septic complications. The combined experience of these 30 patients suggests that corticosteroids are contraindicated in the burn center management of toxic epidermal necrolysis and the Stevens-Johnson syndrome.     

Intravenous immunoglobulins and plasmapheresis combined treatment in patients with severe toxic epidermal necrolysis: preliminary report.

Toxic epidermal necrolysis (TEN) is an acute drug-induced life-threatening disorder characterised by extensive epidermal exfoliation and high rate of mortality. Between October 2000 and April 2003, five severe TEN patients were evaluated using a specific TEN severity-of-illness scale (SCORTEN) and treated for the first time, with a combined therapy using Intravenous Human Immunoglobulins (IVIG) and plasmapheresis. The standardised mortality ratio (SMR) analysis ([Sigma observed deaths/Sigma expected deaths]x100) was applied to establish how IVIG and plasmapheresis treatment could reduce TEN patient mortality. The observed mortality was one out of five patients corresponding to 20%. The expected mortality based on SCORTEN was 3.319 corresponding to 66%. The SMR analysis revealed a 70% reduction in mortality (SMR=0.30; 95% confidence interval, 0.0-0.96). Our series show a low mortality rate (20%) related to the severity of the patients (66% expected mortality). The use of IVIG in association with plasmapheresis has a rational basis and may be effective in severe TEN patients.     

Drug-induced toxic epidermal necrolysis with involvement of the intestinal and respiratory tract. A case report

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.     

Treatment of toxic epidermal necrolysis and a review of six cases

Six cases of drug-induced toxic epidermal necrolysis treated in a burns unit are presented. The mean skin loss was 67.3 per cent of the total body surface area. Two patients developed renal failure and two had ocular symptoms. The mortality rate was 50 per cent, with two patients dying from septicaemia and one from respiratory and renal failure. The diagnosis of toxic epidermal necrolysis can be confirmed by skin biopsy. We recommend that this disease is treated in a burns unit so that both adequate wound care and essential intensive supportive treatment can be given. Antibiotics are indicated only for specific infections such as septicaemia or pneumonia. Steroids have been shown to increase greatly the mortality from septic complications and are not recommended. The mortality ranges from 10 per cent to 70 per cent and bad prognostic factors include increasing age, greater than 50 per cent of body surface skin loss and neutropenia.     

A 12-year retrospective study of non-burn skin loss (burn-like syndromes) at a tertiary burns unit in a developing country

BACKGROUND: A retrospective study of the presentation, etiology, and prognosis of non-burn epidermal loss managed at the Lagos University Teaching Hospital Nigeria over a 12-year period. MATERIALS AND METHODS: Admission records of patients managed for non-burn skin loss were retrieved from the medical records. Demographic details of the patients, the initial diagnosis, final diagnosis, treatment and outcome of treatment was noted. RESULTS: A total of 23 patients were identified, 17 (74%) had idiosyncratic drug reactions. Of this 17, 6 (26%) had Steven Johnson Syndrome, 6 (26%) had Steven Johnson Syndrome/toxic epidermal necrolysis while 5 (22%) presented with toxic epidermal necrolysis. Three of the five patients with toxic epidermal necrolysis died. The age range of patients with idiosyncratic adverse drug reactions was 2-28 years, mean, 10.18+/-1.44 years and male to female ratio of 1:1.83. The body surface area involved ranged from 8 to 78%; mean 26.65+/-6.08%. The agents suspected for the reactions were Co-trimoxazole (41.2%) and combination of Co-trimoxazole, and Fansidar (17.6%). Other conditions seen were two (9%) Staphylococcal Scalded Skin Syndrome, three (13%) had Necrotizing Faciitis, one of whom was HIV positive and died. One (4%) patient presented with pemphigus vulgaris. The presentation and management of the patients was discussed.     

Management of general surgical complications following cardiac transplantation

Between February 1984 and May 1988, 55 patients underwent orthotopic cardiac transplantation at the Brigham and Women's Hospital, Boston, Mass. Basic immunosuppression was accomplished with steroid and cyclosporine therapies. Twelve patients suffered 14 major complications, including perforated ulcer in 3 patients; pancreatitis in 3 patients; pneumatosis coli in 2 patients; and cholecystitis, colonic necrosis, appendicitis, incarcerated umbilical hernia, pancreatic abscess, and toxic epidermal necrolysis in 1 patient each. Aggressive management of the patients included laparotomy in all but 2 patients with mild pancreatitis and the patient with toxic epidermal necrolysis, who was treated as a patient with a severe burn. In all of the patients, there was a resolution of these complications, except in one 59-year-old man with fatal hemorrhagic pancreatitis. Eleven of the 14 complications occurred during the initial hospitalization. The fatal case of pancreatitis was 1 of 5 (9%) operative mortalities in the entire series. Fifty operative survivors have been followed up for an average of 19 months, with four late deaths (8%) related to rejection. The actuarial probability of survival in patients discharged from the hospital was 90% at 12, 24, and 48 months.     

Toxic epidermal necrolysis/Stevens-Johnson syndrome: current trends in management

BACKGROUND: Toxic epidermal necrolysis (TEN) is an uncommon but potentially life-threatening condition that involves sloughing of the skin at the dermoepidermal junction. TEN is a well-recognized syndrome and is part of a range of severe mucocutaneous intolerance reactions, mostly elicited by drugs and/or their metabolites. Reported mortality rates vary widely from 20 to 75%. Several systemic treatment protocols for TEN have been published; however, none has been formally standardized in a randomized controlled trial. The present study documents the current management principles and trends seen in 16 patients admitted with TEN or Stevens-Johnson syndrome over a 5-year period at the Concord Repatriation General Hospital Burns Unit. METHOD: Data were collected by retrospective chart review, and parameters included in the study were patient demographics, causative agents, percentage total body surface area and/or mucosal involvement, complications, treatment and outcome. In particular, dressing choice and documented healing of skin lesions were noted. RESULTS: Sixteen patients were identified, with the beta-lactam antibiotics most commonly implicated as the cause. Complications tended to relate to degree of sepsis and/or mucous membrane involvement. Nanocrsytalline silver dressings, such as Acticoat (Smith & Nephew, Mount Waverley, Victoria, Australia), were used predominantly in more recent cases. CONCLUSION: The shift in dressing choice from traditional Vaseline-impregnated gauze coincided with a general trend towards the use of nanocrystalline silver dressings for superficial burns after 2003. The nanocrystalline silver dressings have shown considerable advantage over previously used dressings, with no adverse reactions noted and good healing of the skin lesions for all patients.     

Lyell syndrome revisited: analysis of 18 cases of severe bullous skin disease in a burns unit.

Over the last few years, understanding of the pathophysiology of toxic epidermal necrolysis (TEN), or Lyell's disease, has substantially increased. However, differentiation of severe bullous skin disease remains a challenge for the clinician, and one that is often complicated by late patient referral. We performed a retrospective analysis of all patients with severe bullous skin disease, admitted between 1997 and 2002 to the Burn Centre, which is an integrated part of the Division for Plastic, Hand- and Reconstructive Surgery at the University Hospital of Zurich, Switzerland. We present an overview of our strategies and of the diagnostic and therapeutic difficulties encountered. The final diagnoses of the 18 patients referred to the unit were as follows: eight cases of TEN, one case of staphylococcal scalded-skin syndrome (SSSS), two cases of generalised drug eruption, one case of acute generalised exanthematic pustulosis and one case of febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta (PLEVA). In two cases, the diagnosis remained unclear. In three cases, paraneoplastic origins were suspected but not demonstrated. The overall mortality rate was 33% (six of 18 patients). Remarkably, all patients with histologically confirmed TEN survived. Six of these patients were successfully treated with intravenous immunoglobulins (IVIG). The most common single causative drug inducing TEN (four cases out of eight) was Phenytoin. Establishing an accurate diagnosis-based on a skin biopsy, harvested at an early stage-is more important than ever, because more specific and effective therapeutic modalities are available. As these potentially life-threatening bullous skin disorders are rare, we recommend, that care be provided by an experienced interdisciplinary team, comprising a dermatologist, or dermatopathologist, an intensive care specialist and a plastic surgeon.     

Toxic epidermal necrolysis: use of Biobrane or skin coverage reduces pain, improves mobilisation and decreases infection in elderly patients

INTRODUCTION: Toxic epidermal necrolysis is a rare disease with high mortality due to generalised infection, sepsis or lung involvement, and requires discontinuation of all potentially triggering medications and intensive care in a specialised burn centre. Apart from wound care with antiseptics, wound coverage may be achieved with a skin substitute; treatments are compared with regard to infection, protein loss, re-epithelialisation and mortality. PATIENTS AND METHODS: Of 14 people with toxic epidermal necrolysis affecting >30% body surface area, eight received daily dressing changes using Lavasept nd six received wound coverage with Biobrane. Demographic data, SCORTEN score, mortality, visual-analog pain scale, mobilisation, time to re-epithelialisation, serum protein, albumin, C-reactive protein and leukocytes, and body temperature were evaluated in all cases. RESULTS: Mean age of patients was 68.0+/-14.8 years, mean body surface area affected was 66.4%, median SCORTEN score was three and overall mortality was 36%. In the Biobrane ompared with the Lavasept control) group, mean pain was significantly reduced (2.9 versus 5.5 on the scale, p<0.05), mobilisation was significantly earlier (walking at 3 days versus 7 days, p=0.003), re-epithelialisation was complete in 12.5 days versus 16 days, and at 9 days there was reduced decrease of serum proteins and significantly lower levels of C-reactive protein and white cells (p<0.05). CONCLUSION: Early wound coverage with synthetic skin substitute such as Biobrane s beneficial compared with conservative antiseptic wound treatment, but mortality rate is not significantly different.     

Leflunomide-induced toxic epidermal necrolysis in a patient with rheumatoid arthritis

IntroductionLeflunomide is an immunomodulating agent with proven efficacy in rheumatoid arthritis. Although its overall safety profile is good, a few cases of toxic epidermal necrolysis have been reported.Case reportThis 36-year-old woman had rheumatoid arthritis that proved refractory to sulfasalazine and methotrexate, which were used successively in combination with symptomatic drugs. Leflunomide was started. A maculopapular rash and a fever developed 2 weeks later. The skin lesions spread rapidly to most of the body, and ulcers of the ocular and oral mucosa appeared. Leflunomide was stopped. Cholestyramine washout and prednisolone (60 mg/day) were given. The skin lesions healed over the next month. Punctate keratitis with keratinization of the cornea led to complete loss of vision.DiscussionThe main adverse effects of leflunomide consist of diarrhea, nausea, liver enzyme elevation, hypertension, alopecia, and allergic skin reactions. A few cases of severe skin reactions such as toxic epidermal necrolysis have been reported. They require immediate discontinuation of the drug and a washout procedure to hasten drug elimination from the body.ConclusionClose monitoring for severe skin reactions is in order when using leflunomide.     

Intense Pulsed Light Treatment of Persistent Facial Hypermelanosis Following Drug-Induced Toxic Epidermal Necrolysis

Background. Cutaneous hyperpigmentation is one of the most cosmetically disturbing sequel of drug-induced toxic epidermal necrolysis. Intense pulsed light is a promising tool for treating some melanocytic lesions.
Objective. The objective was to assess the effect of intense pulsed light in treating post-toxic epidermal necrolysis facial hypermelanosis.
Methods. Two Caucasian men aged 35 and 50 years presented with long-standing (32 and 39 years) severe hypermelanosis of the face after sulfonamide-induced toxic epidermal necrolysis. They were treated by intense pulsed light. Cutoff filters of 550, 590, and 615 nm were employed for five intense pulsed light sessions at 4-week intervals. The treatment was characterized by energy fluence of 25 to 32 J/cm ² , pulse width of 2.2 to 3.2 ms, and double- to triple-pulse mode respecting a 30-ms delay. Before intense pulsed light treatment, and 2 months after the fifth intense pulsed light session, clinical photographs and skin biopsies were performed in combination with quantitative narrow-band remittance spectrophotometry of melanin pigmentation. Patients were clinically followed-up for 8 months after the end of the treatment.
Results. In both patients, clinical, histologic, and spectrophotometric assessments showed an average of 80% decrease in the hypermelanosis. No clinical recurrence of the hypermelanosis developed during the 8-month follow-up after intense pulsed light treatment. No major persistent side effects were experienced, especially hypopigmentation.
Conclusion. Intense pulsed light appears to be effective and safe for treating post-toxic epidermal necrolysis hypermelanosis in Caucasian patients.     

Collagen sheet dressings for cutaneous lesions of toxic epidermal necrolysis

Toxic epidermal necrolysis (TEN) is associated with a significant mortality of 30–50% and long-term sequelae. Treatment includes early admission to a burn unit, where management with precise fluid, electrolyte, protein, and energy supplementation, moderate mechanical ventilation, and expert wound care can be provided. Specific treatment with immunosuppressive drugs or immunoglobulins did not show an improved outcome in most studies and remains controversial. We have treated the cutaneous lesions of seven patients of TEN with collagen sheet dressings and have found a significant reduction in morbidity. The sheets are a one-time dressing, easy to apply and they reduce fluid loss, prevent infection, reduce pain, avoid repeated dressings and gradually peal off as the underlying lesions heal.KEY WORDS: Toxic epidermal necrolysis, Stevens Johnson''s Syndrome, collagen sheet