What is pemphigus?
Pemphigus is a group of skin disorders that cause blisters or pus-filled bumps. Lesions usually develop on the skin, but they can also form in the mucous membranes (soft linings of the 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 the body and have a high risk of infection.
Pemphigus is an autoimmune disorder that can occur in otherwise healthy people. It is sometimes confused with other autoimmune blistering skin diseases such as bullous pemphigoid, lupus erythematosus, and Hailey-Hailey disease.
Pemphigus is not contagious. It is a lifelong condition that can be managed with ongoing medical treatment.
What are the types of pemphigus?
Doctors classify pemphigus into different types based on where and why blisters develop. In most cases, people only get one type of pemphigus.
Types of pemphigus include:
- Pemphigus vulgaris: This type of pemphigus is the most common in the U.S. Blisters essentially always affect the mouth. Some affected people may also form blisters on the skin and in other mucous membranes. These lesions develop in deep layers of the skin. They can be painful and slow to heal.
Pemphigus vulgaris in the mouth (above) and on the legs (below)
- Pemphigus vegetans: This type of pemphigus is related to pemphigus vulgaris. It involves lesions that are thicker and wart-like. These lesions usually form in the areas of the body with skin folds such as the groin and armpit.
- Drug-induced pemphigus: Medications cause blistering with this type of pemphigus. Some drugs that may cause this condition include penicillin and piroxicam. Blisters can develop up to six months after taking the offending medicine.
- Pemphigus erythematosus (Senear-Usher syndrome): This type of pemphigus involves blisters that develop on the upper back, chest, cheeks, and scalp. When lesions form, they are usually red and crusty.
- Pemphigus foliaceus: Blisters develop on the scalp and often the face, neck, and back. Lesions rarely appear in the mouth with pemphigus foliaceus. This type of pemphigus affects the outermost skin layer only. Small blisters may break easily to form crusted lesions that spread to cover large areas of skin.
Pemphigus foliaceus pictured above
- Endemic pemphigus (fogo selvagem): Endemic pemphigus is a form of pemphigus foliaceus that occurs more often in South and Central America, particularly Brazil. This form of the disease often affects multiple family members.
- Paraneoplastic pemphigus: This rarest type of pemphigus develops in people with cancer. Blisters in the mouth that resist treatment may be the first sign. If your doctors diagnose paraneoplastic pemphigus, they will look for signs of a tumor somewhere in your body. Removing the tumor often improves the symptoms of paraneoplastic pemphigus.
How common is pemphigus?
Pemphigus isn’t common. The incidence of pemphigus is different in different locations. However, an estimated 0.75-5 individuals per 1 million people are affected throughout the world every year.
There is a disease called bullous pemphigoid, which has a similar name, but is not pemphigus. Bullous pemphigoid often affects the elderly and can be fatal.
Who is affected by pemphigus?
People of Jewish descent and people from Southeast Europe, India, and the Middle East are at an increased risk of developing pemphigus. The disorder typically develops in people aged 40-60. It is rare in children.
What causes pemphigus?
Pemphigus is an autoimmune disorder. With an autoimmune condition, proteins in your immune system called antibodies attack your body’s own cells.
The particular autoimmune reaction involved with pemphigus causes blisters.
Doctors do not know what causes the immune system reaction of pemphigus. In rare cases, certain medications, including penicillin (an antibiotic) and piroxicam (a nonsteroidal anti-inflammatory drug used for rheumatoid arthritis), can cause the disorder.
What are the symptoms of pemphigus?
The main symptom of pemphigus is blistering skin that may be painful, burn, or sometimes itch.