Pemphigus Vulgaris: BY:-Parth Chauhan (6) B1 Raj Gundaniya (11) B1

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PEMPHIGUS VULGARIS

BY :- Parth Chauhan (6) B1


Raj Gundaniya (11) B1
Overview
• Pemphigus is a rare group of autoimmune
diseases. It causes blisters on the skin and
mucous membranes throughout the body. It
can affect the mouth, nose, throat, eyes,
and genitals. Pemphigus vulgaris is the most
common type of pemphigus.
• Pemphigus vulgaris is the most common
type of a group of autoimmune disorders
called pemphigus. Each type of pemphigus
is characterized by where the blisters form.
Pemphigus vulgaris affects the mucous membranes, which
are found in areas including the:
• mouth
• throat
• nose
• eyes
• genitals
• lungs
Symptoms
The symptoms of pemphigus vulgaris include:

• painful blisters that start in the mouth or skin areas


• skin blisters near the surface of the skin that come and
• gooozing, crusting, or peeling at the blister site
Causes
• Pemphigus is an autoimmune disorder. Normally, your immune system
produces antibodies to fight off harmful invaders, such viruses and bacteria.
But in pemphigus, the body produces antibodies that damage cells of your
skin and mucous membranes.
• The keratinocytes are cemented together at unique sticky spots called
desmosomes. In pemphigus vulgaris, immunoglobulin type G (IgG)
autoantibodies bind to a protein called desmoglein 3 (dsg3), which is found in
desmosomes in the keratinocytes near the bottom of the epidermis. The
result is the keratinocytes separate from each other, and are replaced by fluid
(the blister).
• About 50% of patients with pemphigus vulgaris also have anti-dsg1
antibodies.
Risk factors
• Your risk of pemphigus increases if you're middle-aged or
older. The condition tends to be more common in people
of Middle Eastern or Jewish descent.
• Signs of an infected blister can include:
• the skin becoming painful and hot
• yellow or green pus in the blisters
• red streaks leading away from the blisters
Diagnosed
• A dermatologist will conduct a physical examination of your skin blisters.
They will look for an indicator of the condition called Nikolsky’s sign. A
positive Nikolsky’s sign is when your skin shears off easily when the surface is
wiped sideways with a cotton swab or a finger.
• Direct immunofluorescence of a skin biopsy taken of normal skin near a bulla
shows a linear deposition of C3 and IgG along the basement membrane zone.
• Indirect immunofluorescence of the blood shows linear IgG along the blister
roof on salt-split skin (Many patients do not have detectable circulating
antibodies).
• An enzyme-linked immunosorbant assay (ELISA) for BP 180 and BP 230 is
available.
Treatment
The main aim of treatment is to heal the blisters and prevent new ones forming.
• Steroid medication (corticosteroids) plus another immunosuppressant medication are
usually recommended. These help stop the immune system damaging healthy tissue.
• Common corticosteroids include prednisone or prednisolone. A high dose is usually
needed to control the condition at first.
• You may need to take supplements such as calcium and vitamin D, eat a low-sugar diet,
or take other medications to treat these side effects. 
Additional medications that suppress the immune system. 
• azathioprine
• mycophenolate mofetil

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