Download Treatment of pemphigus

changes seen in epithelial cell junctions
& basement membrane in Pemphigus
Cellular Origin of the DermalEpidermal Basement Membrane
The basement membrane constituents are products of both epithelial and mesenchymal cells.
In vitro modeling of
basement membrane formation clearly shows that, under at least some conditions, the dermalepidermal junction
basement membrane is contributed to by both tissue compartments, and it has been proposed
that differentiated
fibroblasts exist adjacent to epithelial tissues in vivo, which produce basement membrane
components and assist
in basement membrane assembly Of the known basement membrane components, only
laminins 332 (laminin-5) and 311A (laminin-6) are exclusively produced by the epidermis.
Epithelial cells also manufacture most of collagen VII, whereas mainly mesenchymal cells
synthesize collagen IV, nidogen, perlecan, and the laminin α2 chain.
pemphigus
. Pemphigus is a group of rare autoimmune diseases that cause blistering of the skin and
mucous membranes such as in mouth, nose, throat, eyes, and genitals
Types of pemphigus
There are several types of pemphigus. The type of disease depends on what layer in the
skin the blisters form and where they are located on the body. The two main types are:

Pemphigus vulgaris: It is the most common subtype of pemphigus in the United
States and worldwide. It is characterised by blisters and erosions on the skin and
mucous membranes, most commonly inside the mouth.
Pemphigus foliaceus: It is characterised by blistering lesions which usually begin on
face and scalp and later on the chest and other parts of the body. The sores are
superficial and often itchy, and usually aren't painful
Causes of pemphigus
Pemphigus is an autoimmune blistering disease, which means that an individual's
immune system starts attacking its cells in the epidermis, or top layer of the skin, and
the mucous membranes.
It is unclear what triggers the disease, although it appears that some people have a
genetic susceptibility. Environmental agents may trigger the development of pemphigus
in people who are likely to be affected by the disease because of their genes. In rare
cases, it may be triggered by certain medications. In those cases, the disease usually
disappears when the medication is stopped
Treatment of pemphigus
The primary aim of treatment is to decrease blister formation, prevent infections and
promote healing of blisters and erosions. Treatment for pemphigus involves using one
or more drugs. High doses of oral corticosteroids are the mainstay of medical treatment
for controlling the disease. Immunosuppressive drugs are often added to patient's
treatment to minimise steroid use. Severe cases of pemphigus may need plasmapheresis
or intravenous immunoglobulin (IVIG) treatment to reduce the amount of antibodies in
the blood.
.