00:02
Okay, pemphigus vulgaris. Now,
we know that this is an autoimmune
bullous disease, and it's
shown here on the right. It is most
common in patients ages 40-60,
and it's characterized by fragile,
flaccid bullae on normal skin.
00:17
This is because the disease involves
the keratolysis within the epidermis,
rather than the divide between the
epidermis and the dermal layers.
00:25
Patients experience painful erosions,
bleeding from the lesions themselves,
and you'll have a positive Nikolsky sign
because the blisters are so thin-walled
and the disease is in the epidermis that
simply rubbing your thumb on the skin
will lead the skin to separate.
00:41
And it's characterized, typically,
by mucosal involvement,
perhaps of the conjunctiva or the
oral mucosa, or the genital mucosa.
00:50
Pemphigus vulgaris, however,
typically involves the slow
progression over weeks to months,
as opposed to our patient who's describing
symptoms that have progressed over 2 days.
01:00
If you were to biopsy a particular
lesion in pemphigus vulgaris,
you'd find acantholysis, which means
there's a separation of the
bonds between individual keratinocytes.
01:09
. Immunofluorescence, as well, would show
anti-desmoglein immunoglobulin
staining within the epidermis.
01:15
The treatment of configures
for pemphigus vulgaris
typically involves sending
the patients to a burn unit
because it can be a pretty severe disease.
01:21
You're going to use high-dose
intravenous glucocorticoids,
possibly azathioprine as
a steroid-sparing agent,
and you might add on micophenolate
mofetil, as well.
01:30
The title of this slide is not to be
confused with staphylococcal
scalded skin syndrome, which is something
that happens in babies.
01:37
This is staphylococcal toxic shock syndrome,
and it's an acute, multi-organ illness
due to an immune response to a staph
aureus exotoxin.
01:46
Typically, there's some source
of a staphylococcal infection
somewhere in the body.
01:50
Historically, was due to tampons
that had been contaminated,
but oftentimes, nowadays, we'll find it in
skin and soft tissue infection wounds,
post-surgical incisions, sinusitis, burns, etc.
02:01
The specific toxin we're talking about is
TSS toxin-1, TSST-1.
02:07
This is considered a superantigen, which
means that it can activate large numbers of
T cells without even needing any
antigen presenting cells.
02:15
This leads to massive cytokine production
with multiple system effects.
02:20
This can happen within a very short span of
time, as well, within hours to 1 or 2 days.
02:25
Typical manifestations we would
see would be fevers, chills,
macular erythroderma, which is
basically desquamation after
the initial inflammatory phase.
Patients may have diarrhea,
myalgias, mucosal lesions, and
perhaps progress to renal
failure, hepatic failure, or encephalopathy.
02:42
Because of the decreased
systemic vascular resistance,
patients can go into distributive
shock. As you can tell,
these patients are sick. And importantly,
since the infection may actually just
be occurring in some local place
and the toxin is being released systemically,
you may actually have negative
blood cultures, which can
be somewhat paradoxical.
03:01
Nonetheless, we assume that there
is a staphylococcal infection,
so you're going to treat with
things like vancomycin,
Zosyn, and perhaps clindamycin.
03:09
The reason we add clindamycin
is because it suppresses
the synthesis of bacterial toxins,
so it'll hopefully cool down any further
release of the TSS t-1 toxin.
03:19
Now thinking about our patient,
the speed of his illness
is actually fairly consistent. His symptoms
really progressed over 2 days.
03:26
On the other hand, nowhere in
the story are we given any
obvious sources of a
staphylococcal infection,
and the skin findings that our
patient is having seems
more specific than the nondescript,
diffuse erythrodermic reaction of
staphylococcal toxic shock syndrome.
03:41
So, we could certainly go ahead and re-
examine him and look for some occult
nfection that we're missing, but otherwise,
I think we should take a look at
something else on our list.