Pemphigus vulgaris (PV) is a rare autoimmune disease, where the immune system attacks cells found in the outer layer of the skin, as well as cells in the protective lining (mucous membrane) of the mouth, nose, throat, and genitals. This causes blisters to form in the affected tissue. It is unclear what causes these disturbances in the immune system, however certain genes have been linked to an increased risk of PV. The condition mainly affects middle-aged and older people and can lead to problems with sleeping, eating and drinking and weight loss. There is currently no cure for PV, however, treatments can help to keep the disease under control.
Corticosteriods (anti-inflammatory medicines) are usually used for the treatment of PV. Most people will need to take corticosteroids in addition to another medication to help improve the effectiveness and minimise the doses and side effects of corticosteroids. Rituximab is currently being investigated as a treatment option for PV. Rituximab binds specifically to a protein called CD20 located on pre-B and mature B lymphocytes inducing death of these cells. If licensed, rituximab may offer an additional treatment option for patients with PV after failure of systemic steroids and steroid sparing agents to control the disease.
Risankizumab is a drug which is injected into the skin. It works in a unique way by blocking an important molecule in inflammation from a process which allows the body’s immune cells (specifically T-cells) to activate other immune cells and release substances which cause inflammation (cytokines).