Am J Clin Dermatol 2006; 7 (6): 359-368 REVIEW ARTICLE 1175-0561/06/0006-0359/$39.95/0 © 2006 Adis Data Information BV. All rights reserved. Intravenous Immunoglobulin Use in Patients with Toxic Epidermal Necrolysis and Stevens-Johnson Syndrome Nicole Mittmann, 1 Brian Chan, 1 Sandra Knowles, 2 Lidia Cosentino 3 and Neil Shear 1,4 1 Division of Clinical Pharmacology, Department of Medicine, Sunnybrook Health Sciences Centre, Toronto, Ontario, Canada 2 Department of Pharmacy, Sunnybrook Health Sciences Centre, Toronto, Ontario, Canada 3 Talecris Biotherapeutics, Toronto, Ontario, Canada 4 Department of Dermatology, Department of Medicine, Sunnybrook Health Sciences Centre, Toronto, Ontario, Canada Contents Abstract ............................................................................................................... 359 1. Literature Search and Data Analysis .................................................................................. 360 2. Results ............................................................................................................. 362 2.1 Efficacy of Intravenous Immunoglobulin (IVIg) ..................................................................... 362 2.2 Characterization of Toxic Epidermal Necrolysis and Stevens-Johnson Syndrome Patients and IVIg Use ................... 362 3. Discussion .......................................................................................................... 362 4. Conclusions ........................................................................................................ 366 Intravenous immunoglobulin (IVIg) has been proposed as a treatment for toxic epidermal necrolysis (TEN) Abstract and Stevens-Johnson syndrome (SJS). A comprehensive search of the literature was conducted to examine the efficacy and safety of IVIg in TEN and SJS patients. Seventeen relevant articles (14 TEN, 3 SJS) were identified. Only three of the TEN studies and one of the SJS studies were prospective; retrospective studies were the most common study design published. Information regarding disease severity, IVIg use, response, and hospitalization were recorded and cumulated. Aggregate level statistics were calculated. The average IVIg doses used were 0.8 ± 0.4 g/kg/day for a mean duration of 4.0 ± 1.0 days in TEN patients and 0.8 ± 0.2 g/kg/day for 3.4 ± 1.0 days in SJS patients. The clinical experience of IVIg use in TEN and SJS patients was positive in most cases. However, more studies need to be conducted to confirm the benefit of IVIg use in patients with TEN or SJS. Toxic epidermal necrolysis (TEN) and Stevens-Johnson syn- positive Nikolsky sign (epidermal separation induced by gentle drome (SJS) represent different degrees of a severe, acute lateral pressure on the skin surface). [1] Coalescence of lesions is mucocutaneous reaction resulting mainly from use of drugs. [1] frequent, and confluence is widespread in TEN but limited to the TEN and SJS are diagnosed objectively according to a consen- face, neck, and chest in SJS. [1] Necrotic epidermis slides off the sus-derived definition. [2] Clinical features include a macular rash facial region and pressure points on the body, such as the neck and that appears on the face, neck, chin, and trunk but may spread to back, in sheets. [1] According to the consensus-derived definition, [2] the rest of the body. [1] Lesions are flat and tender, resembling SJS is characterized by mucous membrane erosions and blisters on target lesions in appearance but of larger size, and exhibit a <10% of the total body surface area, whereas ‘TEN without spots’