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Pemphigus is a group of rare autoimmune diseases that cause blistering of the skin and mucous membranes (mouth, nose, throat, eyes, and genitals). This condition can occur at any age, but often strikes people in middle or older age. Studies have shown that some populations may be at greater risk for certain types of pemphigus. For instance, people of Jewish descent and those from India, Southeast Europe, and the Middle East are at greater risk for pemphigus vulargis, while pemphigus foliaceus is more common in North America, Turkey, and South America. Pemphigus is a chronic disease which is best controlled by early diagnosis and treatment. Treatment includes steroids to reduce inflammation, drugs that suppress the immune system response and antibiotics to treat associated infections.
There are four main types of pemphigus:
The treatment of pemphigus consists of three phases: control, consolidation and maintenance. During the control phase, intense therapy is given to suppress disease activity until no new lesions appear. In the consolidation phase, drugs and and doses are continued until the majority of the lesion have healed. In the maintenance phase, medication are gradually reduced to the lowest possible dose that will prevent new lesions from appearing.
High-dose oral corticosteroids, such as prednisone or prednisolone, are the main treatment for pemphigus. These are anti-inflammatory medicines that suppress the immune system.Steroids can be applied directly to the affected areas (topically) or be taken by mouth (systemic steroids). High doses of systemic steroids are often required to bring pemphigus under control. To keep the levels of corticosteroid use to a minimum, immunosuppressive drugs and drugs that modify or regulate the immune system are often added to a patient’s treatment. These drugs stop or slow down the immune system’s response to what it sees as an attack on the body. They include:
Once controlled, the steroid is reduced slowly to minimize side effects. Some patients then go into remission; however, many patients need a small maintenance dose to keep the disease under control. If a relapse occurs, renewed efforts to control the disease are undertaken.
People with severe pemphigus that cannot be controlled with corticosteroids may undergo plasmapheresis, a treatment in which the blood containing the damaging antibodies is removed and replaced with blood that is free of antibodies. Such patients can also be treated with IVIg, or intravenous immunoglobulin, which is given daily for 3 to 5 days, every 2 to 4 weeks for 1 to several months. Plasmapheresis and IVIg are both very expensive treatments, since they require large amounts of donated and specially processed blood. Scientists have reported success in treating difficult cases of pemphigus vulgaris with a combination of IVIg and rituximab, a cancer medication.
To learn more about new medications being studied for the treatment of pemphigus, visit the ClinicalTrials.gov web site.