Pemphigus vulgaris is a rare autoimmune disease that causes painful blistering on the skin and the mucous membranes. In an autoimmune disease, a person’s own immune system mistakenly attacks his or her own healthy body.
Pemphigus vulgaris is the most common type of a group of autoimmune disorders collectively 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 the mouth, throat, nose, eyes, genitals, and lungs. This disease usually starts with blisters in the mouth and then on the skin. The blisters sometimes affect the membranes of the genitals.
Although pemphigus vulgaris seems like a somewhat superficial disease, it can be serious. Treatment is essential, and usually entails the use of corticosteroids to suppress the immune system. Without treatment, the condition can cause serious complications, some of which can even be fatal.
According to 2003 guidelines published in the British Medical Journal, before corticosteroids were introduced in the 1950s, the death rate from this disease averaged 75 percent (Harmon, et al., 2003). This has improved dramatically with today’s treatments.
The immune system produces proteins called antibodies. Antibodies normally attack harmful foreign substances such as 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 is not known.
Very rarely, certain medications can cause pemphigus vulgaris. According to the National Institutes of Health, these drugs include:
- penicillamine (chelating agent that removes certain materials from the blood)
- ACE inhibitors (a type of blood pressure medication) (NIH)
Pemphigus vulgaris is not contagious and cannot be transmitted from person to person. It also does not appear to be transmitted from parent to child. However, a person’s genes can put him or her at a higher risk for the condition. In other words, if your parents (or other family members) had or have the condition, you are more likely to develop it as well.
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
The different types of of pemphigus are classified based on the location of the blisters. They include:
The most common type of pemphigus in the Unites States is called pemphigus vulgaris. Blisters usually first appear in the mouth. The blisters do not itch. They can be painful. Blisters may then appear on the skin and sometimes on the genitals.
Pemphigus foliaceus does not 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.
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 and lung problems.
Symptoms of pemphigus vulgaris include:
- painful blisters that start in the mouth
- skin blisters that come and go; the blisters always occur near the surface of the skin
- oozing, crusting, or peeling at the blister site
A dermatologist (doctor who specializes in skin diseases) will conduct a physical examination of your skin blisters. He or she 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 (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.
Treatment is aimed at reducing pain and symptoms and to prevent 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:
- increased susceptibility to infection
- eye problems like cataracts and glaucoma
- increased blood sugar and diabetes
- 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 azathioprine, mycophenolate mofetil, methotrexate, cyclophosphamide, and rituximab.
Antibiotics, Antivirals, and Antifungals
Any of these may be prescribed to prevent other infections.
If your mouth ulcers are severe, you may not be able to eat without pain. You may need to be fed through 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 (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 receive wound treatment. This treatment is similar to the treatment given for severe burns. You may need to receive fluids and electrolytes through a vein (intravenously) if you have lost too much fluid through oozing of the blisters.
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 a dentist to ask about oral care.
Complications can be fatal and severe. They may include:
- skin infections
- sepsis (spread of infection through the bloodstream)
- side effects of medication
Pemphigus vulgaris is life-threatening if not treated. 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 (Harmon, et al., 2003). Some individuals may need to stay on a low dose of the medication for life.