Pemphigus, or bullous diseases, refers to a group of skin disorders that cause blisters in the mouth and elsewhere on the body. Patients develop pemphigus from an irregular autoimmune system response in which antibodies are produced to attack healthy cells in the skin and mucous membranes. Although the specific cause is unknown, this response may be triggered by certain medications or by radiation or ultraviolet light therapy. Pemphigus is most common in patients middle-aged or older, but individuals of any age can be affected.
Symptoms of pemphigus vary depending on its type and severity, though the condition is generally characterized by blisters and open sores, which may be painful and extremely itchy. Blisters in the mouth may affect breathing and swallowing and blisters in other places may adversely affect other normal bodily functions and activities. If left untreated, pemphigus can lead to infections that spread through the bloodstream.
To diagnose pemphigus, several tests may be performed, including a skin biopsy, blood tests, and a test called the Nikolsky’s sign, in which an area of skin that appears to be normal is rubbed to see if the top layers rub off too easily. In forms of pemphigus in which blisters burst easily, there is increased risk of systemic infection and, as a result, a higher rate of mortality.
Although there is no cure for pemphigus, there are many effective treatment options available to manage symptoms and prevent complications from developing. Treatments vary depending on the severity of the condition, but may include corticosteroids, immunosuppressants and biologics. Antibiotics are frequently prescribed to control infections resulting from ruptured blisters. in severe cases, a blood purification procedure called plasmapheresis may be employed.