Final answer:
Pemphigus is managed with systemic corticosteroids, immunosuppressive drugs, and sometimes biological therapies such as rituximab or plasmapheresis and intravenous immunoglobulin for severe cases. The condition requires close monitoring due to potential side effects of the immunosuppressive treatments.
Step-by-step explanation:
Pemphigus is an autoimmune disorder characterized by the production of antibodies against certain proteins in the skin and mucous membranes, leading to blistering. The management of pemphigus typically involves the use of systemic corticosteroids, often as the initial treatment to reduce symptoms and control inflammation. Adjunctive immunosuppressive therapies may be used to help decrease the dose of corticosteroids and to minimize the potential side effects of long-term steroid use.
Additional treatments may include rituximab, a biologic therapy which targets the cells producing antibodies against the skin, as well as other medications like azathioprine, mycophenolate mofetil, or cyclophosphamide which suppress the overall immune response. Plasmapheresis and intravenous immunoglobulin (IVIG) can also be used to remove antibodies from the blood or to provide passive immunity in more severe cases.
Management of pemphigus is a complex process that should involve a multidisciplinary care team, including dermatologists, immunologists, and sometimes other specialists, depending on the organs affected and the severity of the disease. All patients should be monitored closely for potential side effects of the therapies, including infections due to the immunosuppressive nature of the treatments.