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:
Some of the most common types of pemphigus include:
Painful blisters and sores are the main symptoms of pemphigus. However, your symptoms may vary based on the type of pemphigus you have.
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 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.
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 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.
While anyone can develop pemphigus, certain populations are at increased risk. Risk factors for this condition include:
In addition to taking your medical history, doctors rely on several types of exams to diagnose 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:
There is no way to completely prevent pemphigus. However, certain steps may help you reduce and manage flare-ups. You can:
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:
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: