Pemphigus is a rare skin disorder. According to the International Pemphigus and Pemphigoid Foundation, pemphigus is an autoimmune disease or a condition caused by the body attacking itself. Pemphigus is not contagious and is not spread by touching or exposure to body fluids or blood. The American Osteopathic College of Dermatology states pemphigus is characterized by blisters on the skin. Prior to today's drug therapy, many pemphigus sufferers died from infection.
Types of Pemphigus
According to the International Pemphigus and Pemphigoid Foundation, four types of pemphigus exist. Pemphigus vulgaris is the most common form, with the blisters starting in the mouth and traveling down the throat. The mortality rate with pemphigus vulgaris is only 5 to 15 percent.
Pemphigus foliaceus is characterized by crusting or fragile blisters on the face and head but not the mouth. Blisters will eventually spread to the chest and back. The International Pemphigus and Pemphigoid Foundation states the mortality rate of pemphigus foliaceus is much lower than that of pemphigus vulgaris.
The most serious and least common type of pemphigus is paraneoplastic pemphigus. This type of pemphigus usually appears in patients with cancer. Removal of tumors often result in improvement of the pemphigus. Paraneoplastic pemphigus looks different than other forms of pemphigus and can be differentiated by laboratory testing. The Mayo Clinic states the mortality rate of paraneoplastic pemphigus is 90 percent.
According to the International Pemphigus and Pemphigoid Foundation, the last type of pemphigus is benign familial pemphigus. It is a genetic disorder and not an autoimmune disease.
Symptoms
The Mayo Clinic states all types of pemphigus are identified by the presence of blisters. The blisters are easily broken, resulting in open, draining sores that can become infected.
Causes
According to the Mayo Clinic, the exact cause of pemphigus is unknown. The immune system abnormally produces antibodies that attack healthy skin and mucous membranes. The Mayo Clinic states pemphigus has been known to develop as a side effect of certain medications, such as blood pressure medications or medications used to remove metals from the body. This form of pemphigus usually resolves itself after the medication is stopped.
Diagnosis
Pemphigus can be hard to diagnosis. According to the Mayo Clinic, many disorders may have side effects of blisters. The health care provider may assess for a positive Nikolsky sign by gently rubbing an area of skin without blisters with a cotton swab. If the skin separates from the other layers easily, the test is positive. Other diagnostic exams include skin biopsies and blood tests.
Complications
According to the Mayo Clinic, infection of the skin and blood are possible complications. Blood infection or sepsis affects the entire body and can be life-threatening. Paraneoplastic pemphigus sufferers may experience respiratory complications.
Treatment
The Mayo Clinic states the primary goal in treating pemphigus is decreasing signs and symptoms and preventing complications. Treatment should begin as soon as a diagnosis is made. Mild pemphigus may be treated with steroids, immunosuppressants, antibiotics and antifungal medications. According to the Mayo Clinic, severe pemphigus may be treated in the hospital with fluids, intravenous feedings, pain medications and therapeutic plasmapheresis (replacing old plasma with newly donated plasma).
