0:00
The epidermis and
blistering disorders.
This lecture is on pemphigus.
0:07
Pemphigus vulgaris usually presents
on oral and genital mucosa,
and may also involve skin.
The lesions are fragile
bullae that lead
to shallow erosions
that heal very slowly.
Biopsy shows acantholytic
suprabasal cleft.
Direct immunofluorescence of biopsy
reveals epidermal deposits of IgG
and complement in the
intercerllular space.
Indirect immunofluorescence
of serum demonstrates
so-called "intercellular"
antibodies reacting with the cell
membrane of stratified
squamous epithelium.
0:44
Prior to the introduction
of an effective therapy
with oral coritcosteroids
in the 1950s,
the disease had a dismal natural
course, with approximately 50%
mortality rate at two years
and about 100% mortality rate
by five years after the
onset of the disease.
The mortality rate now
is estimated at about 5%,
and death is almost invariably
due to complications
of immunosuppressive therapy.
1:13
Prognosis of pemphigus.
Complete and
long-lasting remissions,
such as no evidence of the disease
and no systemic therapy required
for at least six months,
can occur in about 25%,
50%, and 75% of patients 2, 5,
and 10 years after diagnosis,
respectively.
Patients with mild or
moderate disease and diagnosis
are twice as likely to enter a
long-lasting, complete remission
than those with severe disease.
Patients who respond
rapidly to treatment
are over twice as likely to enter
a long-lasting, complete remission
than those with a slower response.
If not promptly and
aggressively treated,
pemphigus hardens, providing the
opportunity for the spreading
of epitopes and rendering the
disease significantly
more difficult to control.
