Pemphigus foliaceus is part of the pemphigus group of autoimmune diseases. Autoimmune diseases occur when the body’s immune system attacks healthy tissue. In pemphigus foliaceus, the immune system damages skin cells called keratinocytes.
Pemphigus foliaceus (PF) causes blisters, cuts, and crusty spots on the skin. The sores can be painful and unattractive, but PF is a fairly benign medical condition that does not typically cause other health problems.
A chronic, recurring disorder, PF cannot be cured by medical treatment. However, some people go into spontaneous remission, meaning they do not have symptoms for long. PF symptoms can also be managed by treatment.
When a person has PF, their immune system produces antibodies that attach to a protein called desmoglein-1.
This protein is located in sticky spots on skin cells called desmosomes.
When the antibodies attach to this protein, it causes the skin cells to separate from one another, which causes the skin to blister and peel.
Unlike other forms of pemphigus, PF symptoms typically do not involve the mucous membranes, such as the mouth or eyes.
Symptoms of PF include:
- Small fluid-filled skin blisters that typically begin on the face, scalp, or trunk.
- Ruptured blisters that cause sores, pockets, or scars in the skin.
- Scaly, inflamed, painful patches on the skin. These patches occur after blisters burst. Some people may only notice symptoms after the blisters burst.
- Burning, pain, and itching at the site of the blisters.
- Chronic skin infections due to ruptured and irritated blisters.
The blisters and lesions caused by PF can look alarming, but they do not indicate the presence of another condition. On its own, pemphigus is not life-threatening. However, severe blistering leaves the skin vulnerable to serious infections. These infections can become life-threatening if left untreated.
Signs of a skin infection include:
As with many other autoimmune diseases, the cause of PF is still not fully understood.
Researchers believe that both genetic and environmental factors play a role. This means that people who are genetically predisposed to PF may need an environmental trigger in order to develop symptoms.
Some factors that increase the risk of PF, as well as other forms of pemphigus, include:
- Ethnicity and geographic location – people living in Southeast Europe, the Middle East, and India are more vulnerable to pemphigus, as are Ashkenazi Jews.
- Gender – women are more likely than men to develop PF.
- Medications – some anti-inflammatory drugs, particularly those containing sulfur, may trigger PF. When people stop taking these drugs, there is a 50 percent chance that PF symptoms will disappear.
- Sun exposure – PF may be triggered after sun exposure or sunburn.
- Insect bites – in South America, an insect-borne virus called fogo selvagem (FS) can trigger pemphigus.
Pemphigus attacks produce extensive blistering that is hard to miss. Its characteristic pattern on the scalp, face, and trunk makes it unlike most other skin diseases. To confirm suspected pemphigus, a doctor may:
- Perform a biopsy – the doctor will draw a small sample of tissue from a blister through a needle. By viewing the tissue under a microscope, a doctor can confirm the diagnosis.
- Perform a chemical biopsy – with this test, a doctor puts chemicals on the skin to determine what type of pemphigus has caused the blisters.
- Blood work to measure levels of pemphigus antibodies in the blood – to get a sample for this test, blood is drawn from a vein, usually in the arm.
A doctor, usually a dermatologist, will also take a comprehensive medical history. This can help determine whether an environmental factor triggered the pemphigus outbreak, and whether any other autoimmune conditions are present. The doctor may also ask about a family history of autoimmune disorders.
There is no single cure for the condition. Doctors are unsure why some people go into remission and need no treatment, while others continue to fight aggressive outbreaks even with treatment. Once someone has a PF outbreak, they are more vulnerable to a subsequent outbreak.
In some people, outbreaks are continuous. Others experience periods of healing followed by a relapse.
Treatment options for PF include:
- Eliminating triggers for the infection – some people experience an outbreak during times of stress, so reducing stress may help keep symptoms at bay. Eliminating medications that cause PF may also eliminate the blisters.
- Steroid drugs – steroids such as prednisone can slow the course of the disease. However, steroids can trigger serious side effects, such as weight gain, mood changes, and liver problems, so a doctor must carefully monitor steroid use.
- Hospitalization – a severe outbreak of PF may require hospitalization to reduce the risk of infection or treat an infection that has begun to spread.
- Immunosuppressants – these drugs suppress the actions of the immune system, reducing its ability to attack healthy tissue. Immunosuppressants can raise the risk of infections and other illnesses, so people on these drugs may need to make lifestyle changes.
- Antibiotics – either oral or intravenous (IV) antibiotics can fight infections due to PF lesions.
- Anti-inflammatory drugs – these drugs reduce inflammation, and can fight a range of autoimmune diseases, including PF.
- Biologics– these cutting-edge drugs use biological agents to treat the disease in several ways. A drug called Rituximab has shown significant promise, leading to remission in 86 percent of patients in one clinical trial.
People with PF can lead relatively normal lives, but they may need to take time off work or school for treatment or to reduce stress. They may also experience drug-related side effects, anxiety or depression, and self-consciousness about their skin.
PF is part of a group of diseases that also includes:
- Pemphigus vulgaris – the most common form of pemphigus in the United States, pemphigus vulgaris is more likely to affect the mucous membranes, including the mouth.
- Pemphigus vegetans – this form of pemphigus is a variant of pemphigus vulgaris. It causes thick sores under the arms and on the groin.
- IgA pemphigus – caused by an antibody called IgA, this form of pemphigus produces less severe blisters filled with pus.
- Paraneoplastic pemphigus – this rare form of pemphigus occurs in people with cancer and can cause sores in the mouth and eyelids, as well as serious lung problems.