Interventions for toxic epidermal necrolysis

Majumdar S, Mockenhaupt M, Roujeau J-C, Townshend AP

Published Online:

January 21, 2009

Toxic epidermal necrolysis (TEN or Lyell's disease) is a rare life-threatening skin condition. It is probably an immune response triggered by some drugs or infection, which is more likely to happen in people with suppressed immunity. TEN causes extensive blistering and shedding of skin, similar to burns. Drugs used include oral steroids, thalidomide, immunosuppressants and immunoglobulins. This review of trials did not find any reliable evidence for the treatment of TEN. The only trial available used thalidomide, but this trial did not show any benefit from treatment compared against placebo but highlighted increased chances of dying from the treatment. Thalidomide is not safe or effective for the skin condition toxic epidermal necrolysis, but there is not enough evidence to show which treatments are effective.

Abstract (click to read)

Background:

Toxic epidermal necrolysis is a rare condition where a drug reaction induces skin loss, similar to that seen in extensive burns. It is associated with high morbidity and mortality and there is no clear agreement on effective treatment.

Objectives:

To assess the effects of all interventions for the treatment of toxic epidermal necrolysis.

Search strategy:

We searched the Cochrane Skin Group Specialised Register (March 2001), the Cochrane Controlled Trials Register (March 2001), MEDLINE (1966 to December 2001), EMBASE (1980 to December 2001), DARE (4th Quarter 2001) and CINAHL (1982 to October 2001).

Selection criteria:

Randomised controlled trials of therapeutic and supportive interventions that included participants clinically diagnosed with toxic epidermal necrolysis were included.

Data collection and analysis:

Two independent authors carried out study selection and assessment of methodological quality.

Main results:

Only one randomised controlled trial of treatment was identified. This trial compared the effectiveness of thalidomide with placebo and included 22 patients, 12 in the treatment group and 10 in the placebo group. Patients on the treatment arm received thalidomide 200 mg twice daily for 5 days. The main end point was the measurement of the progression of skin detachment after seven days. Other end points were the overall mortality and severity of the disease evaluated with the simplified acute physiology score. The study was terminated as the mortality on the treatment arm was 83% compared to 30% on the control arm (relative risk 2.78, 95% confidence interval 1.04 to 7.40).
No randomised controlled trials of the most commonly used current treatments i.e. systemic steroids, cyclosporin A and intravenous immunoglobulins were found.

Authors' conclusions:

Treatment with thalidomide was not shown to be effective and was associated with significantly higher mortality than placebo. There is no reliable evidence on which to base treatment for toxic epidermal necrolysis, a disease commonly associated with mortality rates of around 30%. More research is required to understand the mechanisms of toxic epidermal necrolysis. International multi-centre studies are needed in the form of randomised controlled trials, to evaluate treatments for toxic epidermal necrolysis, especially those using high doses of steroid and intravenous immunoglobulins.

This record should be cited as:

Majumdar S, Mockenhaupt M, Roujeau J-C, Townshend AP. Interventions for toxic epidermal necrolysis. Cochrane Database of Systematic Reviews 2002, Issue 4. Art. No.: CD001435. DOI: 10.1002/14651858.CD001435

Assessed as up to date:

August 21, 2002

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Primary Review Group:

Skin Group

Health topics:

Skin > Urticaria & other inflammatory disorders

Cochrane Summaries^beta

Independent high-quality evidence for health care decision making

Interventions for toxic epidermal necrolysis

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