Pemphigus vulgarisPemphigus vulgaris is an autoimmine disorder, where the body´s immune system attacks some of the proteins in the skin. Pemphigus usually occurs in middle-aged or older people. This picture shows a close-up of the blistering on the back. Most of the blisters have broken (denuded), which is common since these blisters are fragile.
Pemphigus is a rare autoimmune blistering disorder of the skin. The immune system produces protective antibodies that circulate through our blood and protect us from attack by hostile viruses and bacteria.
In pemphigus however, the antibodies mistakenly consider normal tissues as foreign and attack them. This produces painful raw areas on the skin and mucous membranes (mouth and other moist linings) that will not heal. In some cases, these sores can cover a significant area of the skin. Before modern drug treatments, death from overwhelming infection was the usual outcome, but this is no longer common.
Pemphigus vulgaris is an autoimmune blistering disease, which basically means that an individual's immune system starts reacting against his or her own tissue. Pemphigus vulgaris is the most frequently diagnosed form of pemphigus. Sores and blisters usually start in the mouth. The sores don't always look like blisters. Pemphigus vulgaris is produce by antibodies in the blood that attack the skin directly. The antibodies circulate in the blood, reach the skin and bind to a particular protein found on skin cells. This protein's normal function is to keep the skin cells bound together. When the antibodies bind the protein, the cells fall apart and blistering of the skin and mucous membranes occur.
The building block cells of the epidermis are called keratinocytes. These cells are cemented together at special sticky spots called desmosomes. In pemphigus vulgaris immunoglobulin type G (IgG) autoantibodies bind to a protein called desmoglein 3, which is found in desmosomes in the keratinocytes near the bottom of the epidermis. The result is the keratinocytes separate from each other, and are replaced by fluid, the blister. Pemphigus vulgaris affects people of all races, age and sex. It appears most commonly between the ages of 50-60 years, and is more common in Jews and Indians presumably for genetic reasons.
Pemphigus vulgaris usually starts in middle-aged and older adults, but is can occur at any age. Often, pemphigus not the first disease considered. The first line of defense for all forms of pemphigus is the introduction of oral steroids, usually Prednisone. Cytotoxic drugs such as Imuran and Cytoxan are added to reduce the dose and side effects of the steroids. Other drugs used are injectable gold and Neoral. Mild disease can sometimes be controlled with topical or intralesional steroids. Hydroxychloroquine (Plaquenil) and Dapsone have also have been effective in some cases of pemphigus foliaceus
Systemic corticosteroid therapy is effective in reducing or eliminating the clinical manifestations of pemphigus vulgaris, although doses of prednisone may have to be as high as 400 mg. daily for patients with severe involvement. Topical corticosteroids can be used as an adjunct therapy if the bullae are confined to oral mucosa. Oral or intravenous administration of cyclophosphamide, azathioprine, cyclosporine and methotrexate may have enough beneficial effect to allow reduced dosages of corticosteroids. Even with immunosuppressive therapy, however, almost 10% of patients will die from their disease, either from electrolyte loss, wound infection or treatment complications. Patients with paraneoplastic pemphigus may have to forgo treatment of their vesiculobullous disease until the underlying neoplasm is controlled, although supportive therapy can be instituted.