Is Pemphigus Vulgaris?
Pemphigus vulgaris is a rare autoimmune disease that causes
painful blistering on the skin and mucous membranes. If you have an autoimmune
disease, your immune system mistakenly attacks your healthy tissues.
Pemphigus vulgaris is the most common type of a group of
autoimmune disorders called pemphigus. Each type of pemphigus is characterized
by the location where the blisters form.
Pemphigus vulgaris affects the mucous membranes, which are found
in areas including the:
This disease usually starts with blisters in the mouth and then
on the skin. The blisters sometimes affect the membranes of the genitals.
Pemphigus vulgaris can be dangerous. Treatment is essential, and typically
it involves the use of corticosteroids to suppress the immune system. The
condition can cause serious complications it isn’t treated. Some of these
complications can be fatal.
The death rate from this disease had averaged 75
percent before corticosteroids were introduced in the 1950s. This has
improved dramatically with today’s treatments.
Are the Symptoms of Pemphigus Vulgaris?
The symptoms of pemphigus vulgaris include:
- painful blisters that start in the mouth or skin
- skin blisters near the surface of the skin that
come and go
- oozing, crusting, or peeling at the blister site
Causes Pemphigus Vulgaris?
The immune system produces proteins called antibodies. Antibodies
normally attack harmful foreign substances like bacteria and viruses. Pemphigus
vulgaris occurs when the immune system mistakenly makes antibodies against
proteins in healthy skin and mucous membranes.
The antibodies break down the bonds between the cells, and fluid
collects between the layers of the skin. This leads to blisters and erosions on
The precise cause of the attack by the immune system isn’t known.
Very rarely, certain medications can cause pemphigus vulgaris. These
which is a chelating agent that removes certain materials from the blood
- ACE inhibitors, which are a type of blood
Is at Risk for Pemphigus Vulgaris?
Pemphigus vulgaris isn’t contagious and cannot be transmitted
from one person to another. It also doesn’t appear to be transmitted from
parent to child. However, a person’s genes can put them at a higher risk for
the condition. If your parents or other family members had or have the condition,
you’re more likely to develop it.
Pemphigus vulgaris can affect people of all races, genders, and
ages. However, the condition is more common in the following groups:
- people of Mediterranean descent
- eastern European Jews
- people who live in the rainforests in Brazil
- middle-aged and older adults
Is Pemphigus Vulgaris 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.
Your doctor may then take a biopsy of the blister, which involves
removing a piece of tissue for analysis, and look at it under a microscope to
confirm the diagnosis. The biopsy may be treated in the laboratory with
chemical substances that help your doctor find the abnormal antibodies. Your
doctor can use this information to determine the type of pemphigus.
The different types of pemphigus are diagnosed based on the
location of the blisters. They include:
Pemphigus vulgaris is the most common type of pemphigus in the
Unites States. Blisters usually first appear in the mouth. The blisters don’t
itch. They can be painful. Blisters may then appear on the skin and sometimes
on the genitals.
Pemphigus foliaceus doesn’t cause blisters in the mouth. The
blisters first appear on the face and scalp. Blisters then appear on the chest
and back. The blisters are usually itchy and painless.
Pemphigus vegetans causes blisters that appear on the groin, under
the arms, and on the feet.
A very rare type of pemphigus that occurs in people with some
cancers is called paraneoplastic pemphigus. The blisters and sores may appear
in the mouth, on the lips, and on the skin. This type may also cause scars on
the eyelids and eyes. It can also cause lung problems.
Is Pemphigus Vulgaris Treated?
Treatment is aimed at reducing pain and symptoms and preventing
complications like infection. It involves one or more drugs and other methods.
It may include any of the following:
Corticosteroids and Immune-Suppressing Drugs
A high dose of corticosteroids is the core treatment for the
condition. Common corticosteroids include prednisone
or prednisolone. A high dose is usually needed to control the condition at
These drugs have many side effects, including:
- an increased likelihood of becoming infected
- increased blood sugar
- a loss of muscle mass
- stomach ulcers
- water retention
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.
Once the blisters are under control, the dose may be lowered to the lowest
level needed to prevent new blisters and to keep the side effects at a minimum.
A corticosteroid cream can be used directly on the blisters as well.
To help keep the dose of corticosteroids low, your doctor may
prescribe additional medications that suppress the immune system. These include:
Antibiotics, Antivirals, and Antifungals
Any of these may be prescribed to prevent other infections.
Intravenous (IV) Feeding
If your mouth ulcers are severe, you may not be able to eat
without pain. You may need to be fed through your veins. This involves using an
intravenous (IV) connection.
In very severe cases, a person may undergo a procedure known as
plasmapheresis. This procedure is intended to remove the antibodies attacking
the skin from the blood. During this procedure, the plasma, or fluid part of
the blood, is removed by a device and replaced with donated plasma. This
treatment can be very expensive.
If the blisters are severe, you may need to stay in the hospital
to get wound treatment. This treatment is similar to what’s given for severe
burns. You may need to receive IV fluids and electrolytes if you’ve lost too
much fluid through oozing of the blisters.
The treatment for the blisters may also include:
- numbing lozenges for mouth blisters
- soothing lotions
- wet dressings
- pain medications
- soft food diets
- avoidance of spicy or acidic foods that may
irritate the blisters
- avoidance of too much sun exposure
If the blisters in your mouth keep you from brushing or flossing
your teeth, you may need special oral health treatment to prevent gum disease
and tooth decay. See your dentist to ask them about oral care.
Are the Complications of Pemphigus Vulgaris?
The complications of pemphigus vulgaris can be fatal and severe.
They may include:
- skin infections
- sepsis, or the spread of infection through the
- side effects of medication
Is the Long-Term Outlook for People with Pemphigus Vulgaris?
If left untreated, pemphigus vulgaris can be life-threatening. The
most common cause of death is a severe secondary infection.
Pemphigus vulgaris is a lifelong condition. It cannot be cured.
However, most people go into remission after receiving corticosteroids.
Improvement is usually noticed within days of starting the corticosteroids.
The blisters will heal slowly, especially the ones in the mouth.
On average, the blisters stop forming in two to three weeks. Healing of the
blisters takes six to eight weeks on average. However, full healing can
sometimes take years. Some individuals may need to stay on a low dose of the
medication for life.