What Are the Treatments for Pemphigus?
In the group of autoimmune disorders called pemphigus, layers of skin separate from each other causing painful or itchy blisters on the skin and mucous membranes. Depending on the extent of the skin surface affected, pemphigus can be a very serious or potentially fatal disease. Available treatments can sometimes control but not cure the symptoms. The Mayo Clinic stresses that early treatment provides the best results.
Mild cases of pemphigus that are not widespread throughout the body can be treated without hospitalization. Open wounds and blisters should stay clean and covered to reduce the risk of infection. To reduce blistering and calm the underlying immune dysfunction, doctors usually prescribe corticosteroids and/or immunosuppressant drugs. Corticosteroids can be given orally. According to the Merck Manual, most patients require 1 mg prednisone per kilogram of body weight once per day as the initial dose. When a patient has 7 to 10 days free of new blisters, the dose is tapered off because high levels of systemic corticosteroids can have serious side effects. Lower doses of corticosteroids can be used if immunosuppressants like methotrexate or cyclosporine are prescribed along with them.
Supportive Hospital Care
More serious cases of pemphigus require treatment in a hospital. Wounds on the skin are cleaned and dressed much like burns. To stabilize fluid and electrolyte levels, patients receive intravenous (IV) fluids. In cases where lesions in the mouth or throat make eating too painful, nutrition can be given intravenously as well, and anesthetic lozenges can ease mouth pain.
In addition to these supportive treatments, doctors prescribe drugs to suppress the immune system. Corticosteroids like prednisone and methylprednisone, and immunosuppressant drugs like cyclophosphamide and rituximab can be given intravenously. Some patients undergo plasmapheresis to “clean” their blood and lower the concentration of harmful antibodies so the skin can heal. In this procedure, blood flows out of the body through IV tubing and into a device that separates out the cells from the plasma, which is the liquid portion of the blood that carries the harmful antibodies. The plasma and antibodies are filtered out while the blood cells are returned through another IV line, and donor plasma or IV fluids replace the blood volume lost in the separation procedure.
Drug Side Effects
Some patients enter remission after drug treatment, but that can take years. Meanwhile, doctors try to find the lowest effective dose for the medications because they can have serious side effects. High dose corticosteroids can cause slow wound healing and dangerous infections, osteoporosis, glaucoma and cataracts, diabetes and peptic ulcers. Immunosuppressants can decrease the number of white blood cells, increasing the risk of infection, and can cause liver inflammation, anemia, and nausea and vomiting.
The Mayo Clinic recommends minimizing the potential for further damage to skin by avoiding contact sports and staying out of the sun. Exposure to ultraviolet light and eating garlic, onions, or spicy or acidic foods can cause or worsen blisters. The Mayo Clinic also recommends using talcum powder to prevent clothing or bed sheets from sticking to oozing blisters.
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