Pemphigus Disorders
Pemphigus is a group of rare autoimmune disorders that cause chronic blistering of the skin and mucous membranes. These conditions can be severe, depending on the type. As recently as the 1960s, serious cases of pemphigus were usually fatal, but advances in drug therapy since then can effectively treat the condition and often lead to complete remission.
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Pemphigus Vulgaris
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Pemphigus vulgaris is the most common type of this skin disorder. It nearly always begins with sores in the mouth that then spread to the scalp, throat, interior of the nose, inner eyelids and rectum. These blisters form in the lower layer of the epidermis. The sores may be very painful, and the blistered skin is very fragile and peels easily. Normal areas of skin can develop blisters simply by someone pressing on them.
Pemphigus Foliaceus
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A less severe and less common type of the condition is pemphigus foliaceus. The blisters in this disorder occur close to the skin surface and break, ooze and crust over. They usually appear on the scalp, face, upper chest and back, but not the mucous membranes, unlike pemphigus vulgaris. The sores also are not as painful as those of pemphigus vulgaris, although they are itchyeand vulnerable to infection, which can cause serious complications. Pemphigus foliaceus can occur with systemic lupus erythematosus, an autoimmune disease involving connective tissue.
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Other Types and Related Conditions
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Other forms of pemphigus exist as well, and certain disorders are related to it. Pemphigus vegetans, for instance, causes thick, fast-growing blisters in the lower layer of the skin, usually in the groin and armpits. Paraneoplastic pemphigus can occur in people who have cancer, mainly lymphoma or leukemia. Pemphigoid is another autoimmune blistering disorder, but differs from pemphigus in that it causes blisters underneath the epidermis, and the sores do not break easily.
Possible Causes
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According to the U.S. National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), researchers have not determined a definitive cause for pemphigus disorders. Some people seem to have a genetic susceptibility, and some may be affected by environmental factors. Rarely, medication can cause pemphigus disorders, and the sores usually heal when the medicine is discontinued. Middle-aged and older adults are most likely to get a pemphigus disorder, but it can affect people of any age.
Treatment
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Pemphigus treatment usually involves high-dose corticosteroids, including topical ointments to relieve inflammation and itching, and oral medication, which reduces inflammation and also suppresses the immune system. For more severe cases, a doctor may prescribe immunosuppressive drugs such as azathioprine or cyclosporine. Many people with a pemphigus disorder will achieve complete remission during treatment, while others keep the disease under control with continued medication.
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