Toxic Epidermal Necrolysis

Abstract

Toxic epidermal necrolysis (TEN) represents the most severe drug-related skin condition that is potentially life-threatening with no well-established treatments. The application of corticosteroid therapy is controversial, whereas recently intravenous immunoglobulin (IVIG) therapy is emerging as a promising new method. A severity-of-illness score for TEN (SCORTEN) has gained acceptance in some western countries. In this study, our objectives were to assess the applicability of SCORTEN in Chinese patients with TEN and to evaluate the efficacy of the combination therapy of IVIG and corticosteroid in these patients. We performed a retrospective review of data from 61 patients with TEN treated at our intensive care unit from 2000 to 2010 to assess the performance of SCORTEN. In particular, 55 patients between 2002 and 2010 were grouped as a series to compare the therapeutic effects of corticosteroid therapy and IVIG combined therapy contemporaneously. During this period, 16 patients were administered with corticosteroid therapy and 39 were treated with the combination therapy. An initial dose of 1.5 mg/kg/day of methylprednisolone was given to all TEN patients. The combination therapy was combined with a total dose of 2 g/kg IVIG within 5 days. Areas under receiver operating characteristic curves and Hosmer-Lemeshow statistic were analyzed to illustrate the performance of SCORTEN. The comparison of the efficacy of the two therapies was conducted on the basis of clinical outcomes, standardized mortality ratio (SMR), and survival analysis. The overall actual mortality of patients between 2000 and 2010 was 16% (10/61), statistically insignificantly lower than predicted (24%, SMR = 67.98). Excellent discriminatory power (the areas under the receiver operating characteristic curves: 88.9, 88.2, 90.6%) and good calibration (P = .637, .833, .530) were found in all the groups. In patients admitted between 2002 and 2010, IVIG combined therapy showed a trend toward reducing the mortality rate (13%, SMR = 52.35), whereas corticosteroid monotherapy suggested no such difference (31%, SMR = 123.92). Besides, the cumulative survival rates of the combination therapy were higher at almost all the levels of SCORTEN (P = .002), especially at the score of 5 (P = 3.10 × 10⁻⁷). Compared with corticosteroid alone, the combination therapy arrested progression earlier (P = .013), although it did not significantly lead to a tapering of corticosteroid or a reduction of the time of hospitalization. We concluded that SCORTEN was generally applicable to Chinese patients with TEN. The comparison of the effect indicated that the combination therapy might achieve a better therapeutic effect than the administration of corticosteroid alone, especially in severe TEN patients.

The Effect of Intravenous Immunoglobulin Combined with Corticosteroid on the Progression of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Meta-Analysis

“…Of these, 3 studies recruited patients with SJS only[9, 36, 37], 5 studies chose TEN patients only[26, 31, 38–40] and 7 studies imposed no restrictions about patients recruitment[25, 27, 32, 41–44]. There were 628 individuals included in the analysis, which contained 317 controls applied with steroid only and 311 cases treated by combination of IVIG and corticosteroid.…”

“…Ages varied with a range from below 1 to above 90. Most of these studies used median and range to describe them, however, a few articles[25, 40, 44] reported ages using mean and SD. With regards to sex ratio, it seemed to have a female predominance among SJS/TEN patients.…”

“…There were 17 studies investigated the effect of IVIG on SMR reduction[10, 11, 25–27, 31, 40, 43, 45–53]. Of these, 9 selected TEN patients[26, 31, 40, 45–50] and 8 recruited patients without restriction[10, 11, 25, 27, 43, 51–53], which accumulated 361 cases treated by IVIG within this analysis.…”

“…Of these, 9 selected TEN patients[26, 31, 40, 45–50] and 8 recruited patients without restriction[10, 11, 25, 27, 43, 51–53], which accumulated 361 cases treated by IVIG within this analysis. In terms of therapy, 167 patients were given solo IVIG treatment[10, 11, 46, 47, 49–52], 189 patients were treated with combination of IVIG and steroid[25–27, 31, 40, 43, 45, 53], and 5 patients were treated with IVIG and plasmapheresis[48]. Some articles also reported the time to recover or in hospital with no control to compare with[50, 52, 53].…”

“…Unfortunately, this result was not reliable according to sensitivity analysis ( Fig 3E ). Removal of 9 studies[25–27, 36, 38, 40–43] would reverse the significant overall effect to insignificant one. Of note, these 9 studies were all from Asia.…”