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Paraneoplastic pemphigus

Grant J Anhalt, MD
Section Editor
John J Zone, MD
Deputy Editor
Abena O Ofori, MD


Paraneoplastic pemphigus (PNP) is an often fatal paraneoplastic mucocutaneous blistering disease that is most commonly induced by lymphoproliferative disorders [1]. Paraneoplastic autoimmune multiorgan syndrome (PAMS) is an alternative term used to refer to PNP. This latter term reflects the inclusion of the nonbullous cutaneous eruptions and pulmonary involvement that may develop in the setting of PNP [2].

The pathogenesis, clinical features, and treatment of PNP will be discussed here. Pemphigus vulgaris and pemphigus foliaceus are reviewed separately. (See "Pathogenesis, clinical manifestations, and diagnosis of pemphigus".)


Although it is well-accepted that PNP is a rare disorder, the incidence and prevalence of PNP are unknown. The rarity of the disorder is supported by a review of 100,000 adverse events reports to the US Food and Drug Administration from patients with non-Hodgkin lymphoma and chronic lymphocytic leukemia [3]. The review identified only 12 patients who likely had the disease.

Adults between the ages of 45 and 70 years constitute the most common demographic affected by PNP [4]. However, the disorder may also occur in children [5,6]. Both males and females may be affected. Clear racial, ethnic, or geographic differences in the risk for PNP have not been established.


PNP occurs in association with a variety of neoplastic disorders [7,8]. In a review of 163 cases of PNP reported between 1990 and 2003, the frequency of associated conditions was as follows [7]:

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Literature review current through: Oct 2017. | This topic last updated: Oct 05, 2017.
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