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Treatment of toxic epidermal necrolysis with intravenous immunoglobulin in children,☆☆,

https://doi.org/10.1067/mjd.2002.127249Get rights and content

Abstract

Background: Toxic epidermal necrolysis (TEN) is an acute illness characterized by rapid onset of skin necrosis and high mortality. Standard treatment is primarily aimed at supportive care in a burn unit setting. Objective: We evaluated the outcome of 8 pediatric patients treated for TEN with intravenous immunoglobulin (IVIg) over a 3-year period. Methods: We performed a retrospective analysis of pediatric patients with a diagnosis of TEN between 1999 and 2001, obtained from a computerized database. Results: Mean body surface involvement of 8 patients treated with IVIg was 67%. The average length of hospitalization was 13.6 days, with an average delay in treatment of 3.2 days. The average time to arrest in progression of lesions was 2.1 days and to complete re-epithelialization, 8.1 days. The mortality rate was 0%. The majority of complications were infectious. Conclusion: IVIg is a safe and effective treatment for TEN in the pediatric population. Randomized trials are needed to further evaluate the efficacy of IVIg compared with other modalities. (J Am Acad Dermatol 2002;47:548-52.)

Section snippets

Patients and methods

This retrospective study was approved by the Institutional Review board of the University of Utah Medical Center. We reviewed the charts of 8 pediatric patients who were admitted between June 1999 and March 2001 with a diagnosis of TEN, who were identified from the computerized records of the University of Utah Health Sciences Center, Primary Children's Medical Center, and Intermountain Burn Center. In addition, 3 additional pediatric patients with TEN who were not treated with IVIg between

Results

The demographic and clinical characteristics of patients treated with IVIg are summarized in Table I. There were 4 male and 4 female patients, with a mean age of 8.1 years (range, 22 months to 21 years). Antibiotics were implicated in 4 cases (penicillin in 2 cases, sulfonamides in 2 cases) and anticonvulsants in 3 cases (phenobarbital, carbamazepine, lamotrigine); in one case a specific inciting factor could not be determined. Widespread epidermal necrosis was noted in all patients with mean

Discussion

We present a series of 8 pediatric patients who were treated with IVIg for TEN. Despite significant BSA involvement, all patients survived. In addition, treatment with IVIg resulted in arrest in progression of TEN (mean, 3.2 days) and rapid re-epithelialization (mean complete recovery, 8.1 days), without any toxicity from the drug treatment. These results are in agreement with Viard et al16 who reported the successful treatment of 8 adult and 2 pediatric TEN patients with IVIg (dosage, 0.2-0.75

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      Citation Excerpt :

      IVIG-treated patients demonstrate reduced Fas and FasL in post-treatment skin biopsies.196 There have been numerous SJS/TEN case reports and case series showing benefit from IVIG.197-213 In a small retrospective study of 8 pediatric TEN patients treated with IVIG, all patients survived.201

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    Funding sources: None.

    ☆☆

    Conflicts of interest: None identified.

    Reprint requests: John J. Zone, MD, Chairman, Department of Dermatology, University of Utah School of Medicine, 50 N Medical Dr, Salt Lake City, UT 84132. E-mail: [email protected].

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