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.
Symptoms and Causes
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.
Diagnosis and Tests
How is pemphigus diagnosed?
A doctor diagnoses pemphigus with a medical history and several tests. These tests include:
- Biopsies: A doctor takes samples of tissue and looks at it for the antibodies known to cause pemphigus
- Blood tests: A doctor takes a sample of blood to identify the antibodies that cause pemphigus
- Physical exam: A doctor looks for blisters or skin that easily peels, which are typical signs of pemphigus
Management and Treatment
What are the treatments for pemphigus?
In people with drug-induced pemphigus, stopping the use of the medication that caused the pemphigus usually clears up the condition.
Doctors use medications to treat pemphigus. Treatment should begin as early as possible to stop the spread of blisters. Medicines used to treat pemphigus include:
- Corticosteroids: Medications that reduce inflammation (swelling), delivered by mouth, by injection (a shot) or topically (such as lotions or creams)
- Immunosuppressive drugs: Drugs that manage the autoimmune response, which is what happens when the body attacks itself
- Intravenous immunoglobulin: Healthy antibodies (proteins made by the body to attack foreign substances), given through a needle into a vein, to help destroy the antibodies causing the syndrome
- Plasmapheresis: A procedure that decreases the number of antibodies by removing plasma (liquid) from the blood
Doctors treat non-drug-induced pemphigus in 3 stages. Most people go through all 3 stages of treatment. The stages are:
- Control: High doses of medications control the spread of blisters and begin healing existing ones.
- Consolidation: Steady doses of medications continue healing blisters until most clear up.
- Maintenance: Reduced levels of medications keep new blisters from forming.
What are the complications or side effects of pemphigus treatment?
Your doctor will monitor you regularly, including blood and urine tests, to watch for reactions to medications that treat pemphigus. Serious side effects are possible with some medications. These side effects include:
- Chest pain
- Joint pain
- Upset stomach
- Weight gain
What are the complications associated with pemphigus?
Eating can be difficult for people with pemphigus who develop blisters in the mouth. Over time, painful eating can lead to problems getting enough nutrition. If you have this issue, your doctor may recommend nutritional supplements.
As with any other type of blister, in some cases, infections can develop in pemphigus blisters. If this happens, your doctor may prescribe medications called antibiotics to treat the infection.
Pemphigus can be life-threatening if left untreated.
What can I do to help relieve symptoms of pemphigus?
Steps you can take at home to manage the symptoms of pemphigus include:
- Avoiding spicy foods that can irritate mouth blisters
- Caring for your blisters as directed by your doctor
- Staying out of the sun
- Washing with mild soap
How can you prevent pemphigus?
Doctors do not know what causes pemphigus. There is no known way to prevent it.
Who is at risk of developing pemphigus?
People who are at an increased risk of developing pemphigus include those who are:
- Age 40-60
- From Southeast Europe, India, or the Middle East
- Of Jewish descent
Outlook / Prognosis
What is the prognosis (outlook) for people with pemphigus?
Most people with pemphigus can manage symptoms with ongoing medical treatment. Many people with the disorder live full and active lives.
When should I see a healthcare provider about pemphigus?
Contact your doctor if you develop blisters on your skin or in your mouth that heal very slowly or do not heal at all.
What questions should I ask my doctor?
If you have pemphigus, you may want to ask your doctor:
- How serious is pemphigus?
- What are my treatment options?
- What signs of complications should I look out for?
What resources are available for people with pemphigus?
There are many resources to help support people with pemphigus. Many people with the condition join support groups to share experiences and ways to manage the disorder.