Paraneoplastic Pemphigus. A Life-Threatening Autoimmune Blistering Disease

Abstract

Paraneoplastic pemphigus (PNP), a subset of pemphigus, is a unique autoimmune blistering condition that can affect multiple organs other than the skin. It is a life-threatening disease associated with an underlying malignancy, most commonly of lymphoproliferative origin. The clinical picture may resemble pemphigus, pemphigoid, erythema multiforme, graft-versus-host disease, or lichen planus. The earliest and most consistent finding is a painful, severe, chronic and often recalcitrant stomatitis. Treatment of PNP is difficult. Immunosuppressive agents are required to decrease blistering, and treating the underlying tumor may control autoantibody production. In this review, we included essential diagnostic aspects of PNP and the most useful treatment options in the dermatologist practice.

Keywords: Antibody production; Lymphoproliferative origin; Origen linfoproliferativo; Paraneoplastic pemphigus; Pemphigus; Producción de anticuerpos; Prognosis; Pronóstico; Pénfigo; Pénfigo paraneoplásico; Rituximab.