What 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 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 involves the use of corticosteroids to suppress the immune system. The condition can cause serious complications if 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.
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 go
- oozing, crusting, or peeling at the blister site
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 skin.
The precise cause of the attack by the immune system isn’t known.
Very rarely, certain medications can cause pemphigus vulgaris. These drugs include:
- penicillamine, which is a chelating agent that removes certain materials from the blood
- ACE inhibitors, which are a type of blood pressure medication
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
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 looking 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.
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 first.
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:
- mycophenolate mofetil
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.
The complications of pemphigus vulgaris can be fatal and severe.
They may include:
- skin infections
- sepsis, or the spread of infection through the bloodstream
- side effects of medication
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.