Pemphigus is a rare autoimmune disorder that causes blisters on your skin, genitals, and the inside of your mouth, throat, and nose.
There are several types of pemphigus. They are categorized based on:
- The layer of epidermis affected (outermost layer of skin)
- The parts of the body affected
- The type of antibody (immune system protein) that causes the damage
Some of the most common types of pemphigus include:
- Pemphigus vulgaris: This is the most common type. It forms in deeper layers of your epidermis and mucosal surfaces, which are the membranes that line your airways, lungs, reproductive tracts, and gastrointestinal tract.
- Pemphigus vegetans: Pemphigus vegetans is a subtype of pemphigus vulgaris that primarily affects skin folds, such as your armpits and groin.
- Pemphigus foliaceus: A less common form, pemphigus foliaceus mainly affects the upper layer of your skin and rarely affects inner tissues.
- Paraneoplastic pemphigus: This type affects your skin, lungs, and mouth. It’s associated with an underlying tumor and can lead to severe lung problems.
- IgA pemphigus: Caused by the antibody immunoglobin A (IgA), this type causes clusters or rings of blisters or bumps on the skin.
- Drug-induced pemphigus: In rare cases, certain drugs can trigger pemphigus. These include certain antibiotics, blood pressure medications, and thiol drugs, which are used for lung conditions. Symptoms often go away when you stop taking the medication.
Pemphigus blisters and sores can be painful.
Reproduced with permission from © DermNet dermnetnz.org.
Pemphigus can cause your skin color to change.
Reproduced with permission from © DermNet dermnetnz.org.
Painful blisters and sores are the main symptoms of pemphigus. However, your symptoms may vary based on the type of pemphigus you have.
Pemphigus Vulgaris Symptoms
With pemphigus vulgaris, fluid-filled blisters often develop inside your mouth first. You can then develop blisters on your skin and the mucosal surfaces in your nose, throat, eyes, and genitals. Because the blisters form in deep layers, this type is often painful, and your skin become fragile. In most cases, the blisters go away without scarring, though they may change your skin color in the affected areas.
Pemphigus Foliaceus Symptoms
Pemphigus foliaceus causes blisters to form on your face, scalp, chest, and upper back. These are typically itchy and painful, and they can spread to other parts of your skin. Over time, your skin may become fragile and inflamed and peel off easily. The blisters form crusts as they heal.
Paraneoplastic Pemphigus Symptoms
Typically arising due to cancer, paraneoplastic pemphigus causes blisters on your lips, mouth, tongue, and other mucosal surfaces. This can spread to cause dark patches and red or purple blisters on the skin. Advanced cases may also affect your eyes, lungs, gastrointestinal tract, and other organs. These can lead to blindness, light sensitivity, cough, shortness of breath, and muscular weakness.
IgA Pemphigus
IgA pemphigus causes eruptions of itchy and painful blisters that appear in circles or clusters on your skin. The blisters are fluid-filled that can rupture and form crusts. Most often, the sores appear on your abdomen, chest, or back, though your genitals may also be affected.
Pemphigus occurs when your immune system mistakenly attacks layers of your skin and the mucosal tissues lining your airways, digestive tract, sinuses, and mouth. Antibodies—the substances your immune system uses to fight infection—impact proteins that bind skin cells and mucosal cells to each other. As a result, fluid can build up and the cells become fragile, leading to symptoms.
Researchers aren’t certain what exactly causes the autoimmune disease, but they believe it can occur due to a combination of genetic and environmental factors.
Risk Factors
While anyone can develop pemphigus, certain populations are at increased risk. Risk factors for this condition include:
- Ethnicity: Researchers have found that those of Ashkenazi Jewish and Mediterranean descent have pemphigus vulgaris at higher rates.
- Geography: Pemphigus is most prevalent in certain parts of South America and Tunisia.
- Age: While this disease affects people of all ages, adults aged 50-60 are at the highest risk. However, this can vary based on geographic region.
- Genetics: Some forms of pemphigus are associated with mutations of the HLA genes and other genes.
- Medications: Rarely, certain antibiotics, blood pressure medications, and thiol drugs trigger pemphigus symptoms.
- Cancer: Cancers—often of the lymph nodes, tonsils, or thymus gland—may trigger paraneoplastic pemphigus.
In addition to taking your medical history, doctors rely on several types of exams to diagnose pemphigus:
- Physical evaluation: They will evaluate the affected areas, looking for characteristic signs of pemphigus.
- Biopsy: They may collect a small sample of affected skin and send it to a laboratory for examination under a high-powered microscope.
- Blood tests: They will perform tests on blood samples to detect antibodies associated with pemphigus.
Through these steps, they can rule out other potential causes of similar symptoms, including skin infection, and determine which type of pemphigus you have.
Pemphigus is a chronic (long-lasting) condition with no direct cure. Treatments for pemphigus focus on managing the symptoms when they are present and preventing flare-ups or complications. Specific strategies depend on the type and individual case, with common treatment approaches including:
- Corticosteroids: Your doctor may prescribe oral or topical corticosteroids, such as Deltasone (prednisone) and Orapred (prednisolone), to reduce inflammation and immune system overactivity.
- Rituxan (rituximab): Administered through a vein, this drug acts directly on the immune system to help suppress its activity.
- Immunosuppressants: In some cases, doctors may prescribe immunosuppressant drugs, such as Neoral (cyclosporine) or Otrexup (methotrexate), which help moderate immune function.
- Immunoglobin therapy: Healthy antibodies are administered directly into your blood through a vein to improve symptoms.
- Plasmapheresis: This procedure involves filtering out or diluting the pemphigus-causing antibodies in your blood.
- Antibiotics, antivirals, and antifungals: Because pemphigus blisters can become infected, your doctor may prescribe antibiotic, antiviral, and antifungal drugs. For oral cases, they may offer antibiotic or steroid mouthwashes.
There is no way to completely prevent pemphigus. However, certain steps may help you reduce and manage flare-ups. You can:
- Avoid skin injuries, which may cause blisters to spread.
- Keep any injuries, cuts, or scrapes clean and disinfected.
- Manage stress, since stress can worsen symptoms.
- Eat soft, bland foods and avoid foods that are too hot, hard, or spicy if you have mouth sores.
- Avoid hot or acidic beverages, such as hot tea or coffee and grapefruit juice.
- Maintain good oral hygiene by brushing, flossing, and having regular check-ups and dental cleanings.
- Use soaps and lotions that are gentle on your skin and don’t have fragrances.
- Wear sunglasses, or choose glasses instead of contact lenses if you have eye symptoms.
- Avoid sun or heat exposure, which may trigger pemphigus foliaceus or drug-induced pemphigus.
Pemphigus can lead to serious complications in some cases. Some complications are due to the condition itself, while others arise as side effects of corticosteroid treatment. Conditions often associated with pemphigus include:
- Infection: Blistering on your skin or other tissues can become infected, leading to swelling, discharge, or other symptoms.
- Malnutrition: When pemphigus affects your mouth, it can make eating painful and difficult. This can lead to not getting enough nutrients.
- Osteoporosis: Long-term use of corticosteroids can cause weak or brittle bones and increase your risk of bone fracture.
- Tooth decay: Pemphigus in the mouth can make brushing and flossing painful and difficult, which can increase your risk of cavities and tooth loss.
- Sepsis: This is a severe, life-threatening blood infection caused by viruses, bacteria, or fungi.
- Cushing’s syndrome: This condition can be a side effect of corticosteroid treatments. Cushing’s syndrome occurs due to high levels of the hormone cortisol and has symptoms such as fatty deposits in your face, weight gain in the middle of your body, and loss of fat in your limbs.
- Addison’s disease: Also a potential side effect of corticosteroids, this is when your adrenal glands don’t make enough cortisol. It can cause symptoms like weight loss, loss of appetite, and fatigue.
While there’s no cure for pemphigus, medications and treatments can help manage your symptoms and prevent complications. Prior to current treatments, about half of those diagnosed survived two years. However, with therapy, the rate of mortality has dropped to about 10% or less.
Though the outlook has improved, there’s more to do. Researchers are working on new treatments, such as medications that target the B-cells that produce antibodies. Early clinical tests found these effective in preventing outbreaks of pemphigus.
Still, pemphigus can significantly impact quality of life. Along with the physical symptoms, it can be challenging to take on the mental and emotional strain of living with pemphigus. Several strategies may help you cope:
- Seek support groups for people living with chronic disease.
- Enlist the help and support of loved ones, family, and friends.
- Seek counseling with a mental health provider to address the emotional and psychological effects of the condition.
- Find social media groups or discussion boards devoted to chronic skin conditions.
- Research advocacy organizations, such as The International Pemphigus and Pemphigoid Foundation or the Autoimmune Foundation.
