Pemphigus

Pemphigus is a group of autoimmune skin conditions that cause sores, blisters or fluid-filled bumps to form on your skin. These blisters can also form in your mucous membranes, which are the soft linings of your eyes, nose, mouth, throat and genitals.

The blisters are soft and break open easily to form painful sores. Without treatment, they can spread over large areas of your body and have a risk of infection.

Pemphigus is sometimes confused with other autoimmune blistering skin conditions such as bullous pemphigoid, lupus erythematosus and Hailey-Hailey disease.

Pemphigus isn’t contagious. It’s a lifelong condition that can be managed with ongoing medical treatment.

What are the types of pemphigus?

There are several types of pemphigus based on where and why lesions develop. Types of pemphigus include:

• Pemphigus vulgaris: This is the most common type in the U.S. Blisters always affect your mouth. Some people may have blisters on their skin and in other mucous membranes. These lesions develop in superficial layers of your skin. They can be painful and heal slowly.

Pemphigus vulgaris causes red and white fluid-filled blisters or open sores to form inside of your mouth.

Pemphigus vulgaris causes blisters to form on your skin. A common location for blisters is near your groin and on the skin on your legs.

• Pemphigus vegetans: This type is similar to pemphigus vulgaris but causes thicker lesions. These lesions usually form in areas of your body with skin folds such as your groin and armpit.
• Drug-induced pemphigus: Medications can cause blistering. Some drugs that cause this condition include antibiotics and blood pressure medication. Blisters can develop months after taking the medicine.
• Pemphigus erythematosus (Senear-Usher syndrome): This type is an overlap syndrome with lupus that causes blisters to develop on your upper back, chest, cheeks and scalp. When lesions form, they’re usually red and scaly.
• Pemphigus foliaceus: This type causes blisters to develop on your scalp, face, neck and back. Lesions rarely appear in your mouth. This type affects your outermost skin layer only. Small blisters may break open easily to form crusty lesions that spread to cover large areas of skin.

Pemphigus foliaceus causes red to purple blisters to form most often on your back in groups that affect the outside layer of your skin. These blisters can easily spread to cover a large area of your skin.

• Endemic pemphigus (fogo selvagem): This is a form of pemphigus foliaceus that occurs more often in South and Central America, particularly Brazil.
• Paraneoplastic pemphigus: This is the rarest type of pemphigus that develops in people diagnosed with cancer. Severe blisters form in your mouth. If your healthcare provider diagnoses paraneoplastic pemphigus, they’ll look for signs of cancer somewhere in your body.

Who does pemphigus affect?

Pemphigus can affect anyone. It’s most common among people between the ages of 40 and 60.

Specific geographical regions of the world have a higher number of cases, including:

• Southeast Europe.
• India.
• The Middle East.
• Tunisia.
• Brazil.

How common is pemphigus?

Pemphigus isn’t common. An estimated 1 to 5 out of every 1 million people receive a pemphigus diagnosis throughout the world each year.

How does pemphigus affect my body?

Pemphigus causes blisters and sores to form on your skin. These lesions form quickly and can last for years, with new blisters appearing in the same area of your skin after one blister goes away. These lesions can be painful and cause additional symptoms like infections. Contact your healthcare provider if you have blisters that are widespread across your body, as they could be life-threatening.