0:00
Hello, my name is Dr. Giuseppe Ficarra.
I'm the Director of the Reference Center
for the Study of Oral Diseases in Florence.
And this is the second part,
we'll discuss pemphigus vulgaris
which is another fearsome disease.
0:17
Pemphigus represents
a group of blistering diseases
of skin and mucosal membrane.
There are several entities
included in the group.
Pathogenesis relates
to the action of autoantibodies
directed against transmembrane
adhesion molecules
which are constituents
of the keratinocytes desmosome.
These molecules are called desmogleins,
their destruction causes
epithelial disruption.
0:51
There are several types of pemphigus.
The most common entity is pemphigus vulgaris,
which although a rare disease
can involve the skin and the oral cavity.
Pemphigus foliaceus involves
only the skin
and typically seen in an endemic form
in certain geographic area
such as such South America,
for example, Brazil.
It includes several subtypes.
Paraneoplastic pemphigus
associated with a neoplastic disease
usually non Hodgkin lymphoma,
it will be discussed in more detail
later on.
1:32
The pemphigus vulgaris
is a quite rare disease,
and overall, its incidence
is between 0.076 cases to 5 cases
per 100,000 persons per year.
Likely then from this data
you can see that this is a very rare disease.
It shows preference for women.
