Phemphigus Vulgaris

Patient Education

What Is Pemphigus Vulgaris?

Pemphigus Vulgaris is a rare autoimmune disease that causes painful blistering on the skin and the mucous membranes. 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.

What Causes Pemphigus Vulgaris?

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. Very rarely, certain medications can cause pemphigus Vulgaris. According to the National Institutes of Health, these drugs include:

• penicillamine
• ACE inhibitors

What are Types of Pemphigus?

The different types of pemphigus are classified based on the location of the blisters. They include:

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

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.

Pemphigus vegetans

The blisters appear on the groin and under the arms and on the feet.

Paraneoplastic Pemphigus

What Are the Symptoms of Pemphigus Vulgaris?

• 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 Diagnosed?

a physical examination of skin blisters.

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 to find the abnormal antibodies.

How Is Pemphigus Vulgaris Treated?

Corticosteroids and Immune-Suppressing DrugsA high dose of corticosteroids is the core treatment for the condition. Common corticosteroids include prednisone or prednisolone. 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. Corticosteroid cream can be used directly on the blisters as well.

These include azathioprine, mycophenolate mofetil, methotrexate, cyclophosphamide, and rituximab.

Plasmapheresis In very severe cases, a person may undergo a procedure known as plasmapheresis..

• 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