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Pemphigus Vulgaris

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.
What Causes Pemphigus Vulgaris?

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)

What Are the Symptoms of Pemphigus Vulgaris?

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

How Is Pemphigus Vulgaris Treated?

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
osteoporosis
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.
IV Feeding

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.