00:02
Stevens-Johnson syndrome. So let's
talk about that for a moment.
00:06
Essentially, Stevens-Johnson
syndrome is most often
a drug-mediated disease process
that lies on a spectrum with, at one end,
erythema multiforme, which
can be caused by either
medications or sometimes infections,
then Stevens-Johnson syndrome,
medications or sometimes infections,
then Stevens-Johnson syndrome,
It is a T cell-mediated attack on
keratinocytes. And again, if the attack is
on the keratinocytes themselves,
rather than the basement membrane,
you're going to expect very thin-walled
bullae, which are very likely to rupture.
00:39
You're not going to have those
tense bullae. As I mentioned,
most often, it's caused by medications,
but 70% of the time,
though, occasionally, its associated with
mycoplasma or other types of infections
in < 30% of cases.
00:54
SJS, unlike something like
bullous pemphigoid,
you're definitely going to have
some constitutional symptoms:
fevers, myalgias, malaise, etc.
01:04
nd this is a fairly rapidly
progressive disease, as well.
01:07
This isn't something that's going to be
indolent and take months to develop.
01:11
This could really occur within a
matter of a few days to a week.
01:15
So, rapid progression of
erythematous macules,
then erythroderma, which
means total body redness,
and then your bullae are going to form,
you're going to start seeing those
mucosal lesions, etc.
01:26
In this case, because that skin,
the wall of that blister, is so thin,
Nikolsky sign will be positive.
01:33
Just rubbing your thumb along an area
of skin that appears to be affected
is going to lead that skin
to peel right off.
01:41
90% of cases are going to have mucosal
involvement, whether it's the oral mucosa,
the lips, the conjunctiva, or the genitalia.
01:49
And as you can imagine, with a disease
that involves such an extensive area
of skin as well as mucosal areas with
all these systemic symptoms,
it does have a pretty high morbidity
and mortality associated with it.
02:00
You're very susceptible to infections,
electrolyte imbalances due
fluid shifts and being unable to hold on to
all the water in your body, hypovolemia,
and potentially, septic shock.