Pemphigus is a life-threatening autoimmune blistering disease affecting the skin and mucosa. Patients with severe pemphigus require long-term treatment with corticosteroids and other immunosuppressive drugs, which can lead to serious adverse events. Rituximab, a monoclonal antibody directed against the CD20 antigen of B lymphocytes, has been demonstrated to be effective in recalcitrant and life-threatening pemphigus.