Toxic epidermal necrolysis and intravenous immunoglobulin: a review

Toxic epidermal necrolysis (TEN, Lyell's syndrome) is a rare, life-threatening hypersensitivity reaction to certain medications, such as sulfonamides, antibiotics, non-steroidal anti-inflammatory drugs, and anti-convulsants. The incidence of TEN is 0.4-1.2 cases per million per year in the general population and 1 case per thousand per year in the HIV+ population. It is characterized morphologically by ongoing apoptotic keratinocyte cell death that results in the separation of the epidermis from the dermis. TEN carries a mortality of upwards of 40%, with the vast majority of patients succumbing to sepsis or multiorgan failure. Recent insights into the pathogenesis of TEN revealed that keratinocytes undergo Fas-FasL mediated apoptosis. No specific treatment for TEN exists to date. Attempts have been made to decrease mortality in TEN patients through supportive care and adjuvant therapies. Since 1988, intravenous immunoglobulin (IVIG) has been shown to interfere with the interaction of Fas and FasL, and can be used as a treatment for TEN. This paper reviews the recent studies in the literature that have looked at the use of IVIG to treat TEN.