IVIG Treatments for SJS / TEN
ABSTRACT
Stevens-Johnson syndrome (SJS) and toxic
epidermal necrolysis (TEN, Lyell’s syndrome) are now considered to be distinct clinical entities within a
spectrum of adverse cutaneous drug reactions of increasing severity based on their surface of skin
detachment. Within this spectrum, SJS which can be considered as a minor
form of TEN is characterized by less than 10%
body surface area of skin detachment, and an average reported mortality of 15%,
whereas TEN is characterized by more than 30% skin detachment, and an
average reported mortality 25-35%.
Both SJS and TEN are characterized
morphologically by the rapid onset of keratinocyte cell death by
apoptosis,
a process that results in the separation
of the epidermis from the dermis. Recent evidence is supportive of a role for inflammatory cytokines and the
death receptor Fas and its ligand FasL in the pathogenesis of
keratinocyte apoptosis during TEN. This Fas-mediated
keratinocyte apoptosis that is the last step culminating in epidermal detachment in TEN can be inhibited
in vitro by antagonistic monoclonal
antibodies to Fas, and by intravenous immunoglobulins (IVIG) which have been
shown to contain natural anti-Fas antibodies. Consequently, over the last few years,
numerous case reports and 9 non-controlled clinical studies containing 10 or more patients have analyzed the
therapeutic effect of IVIG in TEN. Taken together, although each study has its potential biases, 7 of 9 such
studies point towards a benefit of IVIG used at doses greater than 2 gkg on the mortality associated with TEN.
These studies should serve as the basis for designing an appropriate prospective trial or for conducting a
metaanalysis in the near future, in order to determine the therapeutic
efficacy of IVIG in TEN.
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in Children With Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: Seven Cases and
Review of the Literature
Stevens-Johnson syndrome (SJS) and toxic epidermal
necrolysis are the most severe cutaneous reactions that occur
in children. Off-label use of human intravenous
immunoglobulin (IVIG) has been reported in a number of
autoimmune and cell-mediated blistering disorders of the
skin, including severe cutaneous drug reactions.
We review 28
previous reports in which IVIG was used in pediatric patients
with SJS and toxic epidermal necrolysis and discuss our
experience in 7 children with SJS, in whom no new blisters
developed within 24 to 48 hours after IVIG administration and
rapid recovery ensued.
IVIG seems to be a useful and safe therapy
for children with severe cutaneous drug reactions.
Well-controlled, prospective, multicenter clinical trials are
needed to determine optimal dosing guidelines and to compare
the efficacy and safety of IVIG with other potentially
effective modalities.
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