Paraneoplastic pemphigus (PNP) is a debilitating and lethal adult and pediatric autoimmune blistering
skin disease that occurs in the setting of a known or occult neoplasm. It is characterized by painful mucosal erosions
that are often resistant to therapy. These clinical characteristics, in addition to positive immunohistochemistry
findings make the diagnosis of PNP. The ability to recognize and diagnose PNP has dramatically improved in the
last two and half decades. However, the mortality remains 75-90% with a mean survival of less than 1 year. The
disease typically progresses from localized skin manifestations to multiple organ failure despite treatment with
high dose corticosteroids and immunosuppressives. Because PNP has a low incidence and prevalence, information
on treatment and management strategies has depended on a limited number of case reports and case series. The
rarity of the disease hampers the ability to conduct large-scale clinical trials as well as investigations into the
pathophysiology of PNP. In this article, we review clinical presentation, diagnosis, management, treatment, and
pathophysiology of PNP.
Keywords: Autoantibodies, blistering disease, dermatology, desmoglein, paraneoplastic pemphigus, pemphigus.
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