Next up, pemphigus vulgaris.
So, pemphigus vulgaris,
in contrast to bullous pemphigoid.
It's important to try and make sure
we separate those 2 disease process.
Pemphigus vulgaris, much more significant,
and potentially can be
a fatal disease process.
Both of them are autoimmune diseases,
but where the immune-mediated
attack occurs is very important.
It's most common in somewhat younger
ages, those 40-60 years of age,
with an equal predilection for
men and for women.
And it is essentially caused
IgG antibodies that are attacking
the keratinocytes within the epidermis.
So, since the epidermis itself is being
attacked, it's going to break up and be thin,
and for that reason, you're going
to have fragile, flaccid bullae
on normal skin. Oftentimes,
when a patient presents,
you may not see any bullae whatsoever,
even though this is a lecture
about bullous diseases,
the bullae are so thin-walled
that they're very
prone to rupture before
you even see the patient.
These lesions are painful,
they may be bleeding
and definitely, you're going to
have a positive Nikolsky sign
by just, again, rubbing that skin. You'll
see the skin slough right off.
Extensive mucosal involvement,
just like we saw with
Patients may report odynophagia,
pain with swallowing,
epistaxis, through the nosebleeds and these
ulcerative lesions within the nasopharynx,
conjunctiva may be involved.
And as you might imagine, this can
be complicated by secondary infections.
Malnutrition can occur, mostly because
they have trouble eating due to all that
discomfort if they don't get
some parenteral nutrition.
If you were to biopsy any one
of these lesions, and that
is often recommended to ensure
that you've got the right diagnosis,
you'll see acantholysis, which just
simply means separation and
well, peeling apart of the different
keratinocytes in the skin.
Immunofluorescence will identify
immunoglobulins that I mentioned. Again,
you'll find them right within the epidermis
rather than at the epidermis-dermis junction.
And of course, with this disease since
it is such a severe disease with a
relatively high fatality rate, you're
going to have to send that
person to a specialized burn unit
where they can really manage all those
potential complications of having
your skin not functioning.
They're oftentimes treated with high-dose
at least initially, to control the
disease process, and then over
time, you may add on some steroid-sparing
agents such as azathioprine
or mycophenolate mofetil.