Pemphigus Vulgaris

Overview

Pemphigus is a group of rare skin disorders that cause blisters and sores on the skin or mucous membranes, such as in the mouth or on the genitals.

The two main types are pemphigus vulgaris and pemphigus foliaceus. Pemphigus vulgaris usually starts in your mouth. It can be painful. Pemphigus foliaceus affects the skin and tends to be more itchy than painful. Pemphigus can occur at any age, but it’s most often seen in people who are middle-aged or older.

Pemphigus is not to be confused with bullous pemphigoid, another blistering skin condition. Usually a chronic condition, pemphigus is best controlled by early diagnosis and treatment. Treatment may include medications and therapies similar to those used for severe burns.

Symptoms

Pemphigus is characterized by blisters on your skin and mucous membranes. The blisters rupture easily, leaving open sores, which may ooze and become infected.

The signs and symptoms of the two main types of pemphigus are as follows:

Pemphigus vulgaris. This type usually begins with blisters in your mouth and then on your skin or genital mucous membranes. The blisters typically are painful, but don’t itch. Blisters in your mouth or throat may make it hard to swallow and eat.
Pemphigus foliaceus. This type doesn’t usually affect mucous membranes. And the blisters tend not to be painful. This condition may affect any skin, but most blisters are on the chest, back and shoulders. The blisters cause the skin to be crusty and itchy.

When to see a doctor

See your doctor if you develop blisters inside your mouth or on your skin. If you’ve already been diagnosed with pemphigus and are receiving treatment, see your doctor if you develop:

• New blisters or sores
• A rapid spread in the number of sores
• Fever, redness or swelling, which may indicate infection
• Chills
• Weakness or achy muscles or joints

Causes

Pemphigus is an autoimmune disorder. It’s not contagious. In most cases, it’s unknown what triggers the disease.

Normally, your immune system attacks foreign invaders, such as harmful viruses and bacteria. But in pemphigus, your immune system mistakenly produces antibodies that attack healthy cells in your skin and mucous membranes.

Rarely, pemphigus develops as a side effect of medications, such as certain blood pressure drugs. This type of pemphigus usually disappears when the medicine is stopped.

Risk factors

Your risk of pemphigus increases if you’re middle-aged or older. People of Jewish ancestry have an increased incidence of pemphigus vulgaris.

Complications

The open sores of pemphigus make you highly vulnerable to infection, which, if it spreads to your bloodstream, can be fatal. Possible complications of pemphigus include:

• Infection of your skin
• Infection that spreads to your bloodstream (sepsis)
• Gum disease and tooth loss, if you have blisters in your mouthMedication side effects, such as high blood pressure and infection
• Death from infection