Treatment of Toxic Epidermal Necrolysis With High-Dose Intravenous Immunoglobulins

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Abstract
TOXIC EPIDERMAL necrolysis (TEN, also known as Lyell syndrome) is an acute and life-threatening mucocutaneous disease that is almost always drug related. The disease occurs at an estimated incidence of 0.4 to 1.2 cases per million, and most frequently as a result of sulfonamide, anticonvulsivant, or nonsteroidal anti-inflammatory drug use.1,2 It is a consequence of extensive keratinocyte apoptosis that results in the separation of large areas of skin at the dermoepidermal junction, producing the appearance of scalded skin. This extensive cell death also results in mucous membrane detachment and contributes to the characteristic symptoms of TEN that include high fever, extreme skin pain, anxiety, and asthenia.1,2 Clinically, cases of TEN with the most extensive skin detachment are associated with the highest mortality, and when large case series are compiled, they point to an average mortality rate ranging between 25% and 35%.3-5 However, studies analyzing elderly patients6 indicate a rate of mortality averaging 50%.