|
Toxic epidermal necrolysis and intravenous immunoglobulin: a review.
Semin Cutan Med Surg. 2006 Jun;25(2):91-3.
Trent J, Halem M, French LE, Kerdel F.
Department of Dermatology and Cutaneous Surgery, University of Miami School of Medicine, Miami, FL.
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.
|
