Pemphigus is a serious blistering skin condition, the most common form of which is called pemphigus vulgaris. When large areas of the skin are involved, a large amount of fluid and protein can be lost through the skin and can be life-threatening.
These blisters tend to rupture or tear very easily and tend to look more like open or eroded areas of skin that variably crust over with time. Mucous membranes of the mouth, lips, and genitalia are often involved, though any area of the body can be affected.
Pemphigus is considered an autoimmune disease in which the body creates antibodies and triggers an inflammatory reaction to proteins that naturally occur in the skin. Blisters are formed due to loss of the connections that hold skin cells together, leading to the formation of the blisters. This process can occur spontaneously or can be linked to the use of certain medications and other medical conditions, such as cancer.
Treatment needs to be aggressive by a health care professional to ensure that a flare is adequately controlled.
Antiflammatory agents and suppression of the immune system: Systemic corticosteroids are used in the initial treatment. Once the condition has shown a response to therapy by decreasing in severity, one can transition into a different systemic immunosuppressant. These other immunosuppressive medications have classically included mycophenolate mofetil, azathioprine, and cyclosporine, to name just a few, and can be administered orally, via injection, or intravenously. Newer medications for pemphigus treatment fall into the category of biologic therapy and often have an immunologic target. One such example is rituximab, a monoclonal antibody directed against the CD20 antigen protein on B-lymphocytes. Other medications are currently being worked on to target a wider range of antigens, potentially leading to greater treatment options in the future.
1. Huang A, Madan RK, Levitt J. Future therapies for pemphigus vulgaris: Rituximab and beyond. J Am Acad Dermatol.2016;74(4):746-753; PMID: 26792592.