Contemporary management of pemphigus

Introduction: The two major pemphigus variants, pemphigus vulgaris and pemphigus foliaceus, are characterized by autoantibodies against desmoglein (Dsg) 3 and Dsg1, respectively, structural components of epidermal desmosomes. In pemphigus, autoantibody binding leads to blisters and erosions on skin and surface-close epithelia, evolving into disfiguring and life-threatening symptoms. Although rare, pemphigus represents an important dermatological condition. Due to the obvious disease burden, the molecular identification of the target antigens, and the evident pathogenicity of autoantibodies, pemphigus is an interesting model to explore novel therapeutic approaches. Areas covered: The authors provide an overview of treatment options based on data from prospective and retrospective studies as well as larger case series. Currently ongoing prospective studies and upcoming and potential treatment approaches are discussed. Expert opinion: While systemic corticosteroids combined with other immunosuppressants are still the therapeutic mainstay of pemphigus, novel strategies such as rituximab, immunoadsorption, and high-dose intravenous immunoglobulins have a definitive place in the management of the high number of patients refractory to conventional treatment. The recently published international consensus on definitions of outcomes and end points facilitates urgently needed randomized controlled clinical trials and may foster proof-of-concept studies for novel therapies that are based on growing insights into the pathophysiology of pemphigus.