What is pemphigus?

Pemphigus is a rare disease and autoimmune disorder that is characterized by group of skin disorders that cause blisters and sores on the mucous membranes of the nose, eyes, mouth, and genitals. It is not contagious. There are two main types of pemphigus: pemphigus vulgaris and pemphigus foliaceus. Pemphigus vulgaris is the most common type. Pemphigus can occur at any age, however, people who are middle aged or older are most often affected. It is estimated that about 1 in 20,000 to one in a million people develop some form of pemphigus each year.

The main signs or symptoms of pemphigus include blisters on your skin and mucous membranes. These blisters can rupture easily which result in open sores. These open sores are susceptible to becoming infected. The signs and symptoms of pemphigus vary depending on the type. Symptoms of pemphigus vulgaris include blisters in your mouth which rupture. These blisters may also appear on the mucous membranes of your genitals. These blisters usually painful, but they do not itch. The symptoms of pemphigus foliaceus include blisters that usually appear on your face and scalp. These blisters may also appear on your chest and back. They are usually not painful, but may be itchy.

Because pemphigus is an autoimmune disorder, it is unknown as to what triggers the disease. The risk factors for developing pemphigus include being middle-aged or older, being of Eastern European Jewish descent or Mediterranean descent, living in the rain forests in Brazil, being exposed to pesticides and some medications such as calcium channel blockers, having lymphoma, leukemia, and other cancers.

There are some potential complications that may occur as a result of pemphigus such as an infection of your skin, an infection that spreads through your bloodstream (aka sepsis), or side effects of medications such as an increased risk of infection. It is very rare for anyone to die from the infection.

Treating pemphigus involves reducing the signs and symptoms and preventing any potential complications. In order to treat pemphigus, your doctor may recommend one of the following medications: corticosteroids (either pills or injections), corticosteroid creams, immunosuppressants (drugs that suppress the immune system), antibiotics for infections (such as antiviral and antifungal medications), biological therapies, or stopping the use of any medications that may have triggered the condition. If pemphigus has spread throughout the body, you may need to be hospitalized in which case you may be treated with fluids, intravenous feeding, anesthetic mouth lozenges, and therapeutic plasmapheresis.