00:02
Now, let's talk about the actual disease
that our patient ultimately has;
bullous pemphigoid. We've talked a little
bit about it already, but let's
just highlight a few key points. It is,
like pemphigus vulgaris,
an autoimmune bullous disease.
Most common in
men and women over the age
of 60. And in this case,
the antibodies are directed against
proteins at the epithelial
basement membrane. Again, at that
dermal-epidermal junction.
00:28
Regions are often pruritic, initially,
urticarial plaques,
and ultimately, over time, you'll start
to see these tense bullae
appear on normal or on erythematous skin,
and it can take months for those
things to develop.
00:42
These patients will have a
negative Nikolsky sign.
00:45
Rubbing on the skin will not lead
the skin to slough off.
00:49
Patients don't have constitutional symptoms,
as was the case in our patient.
00:53
Very infrequently will you see the
mucosal tissues involved,
and if you were to perform a biopsy, you
would see immunofluorescence
with linear IgG or C3 staining along that
basement membrane, at that junction
we talked about.
01:08
Treatment is going to be topical
or, more likely,
oral glucocorticoids, which you may need
to prescribe for a number of months.
01:15
And again, you're going to use those
steroid-sparing agents as soon as you
can with azathioprine or
micophenolate mofetil.