Paraneoplastic Pemphigus/Paraneoplastic Autoimmune Multiorgan Syndrome: Part II. Diagnosis and Management

Simo Huang; Hannah J Anderson; Jason B Lee

doi:10.1016/j.jaad.2023.08.084

Paraneoplastic Pemphigus/Paraneoplastic Autoimmune Multiorgan Syndrome: Part II. Diagnosis and Management

J Am Acad Dermatol. 2023 Sep 13:S0190-9622(23)02685-3. doi: 10.1016/j.jaad.2023.08.084. Online ahead of print.

Authors

Simo Huang¹, Hannah J Anderson¹, Jason B Lee²

Affiliations

¹ Department of Dermatology, Thomas Jefferson University Hospitals, Philadelphia, Pennsylvania.
² Department of Dermatology, Thomas Jefferson University Hospitals, Philadelphia, Pennsylvania. Electronic address: jason.lee@jefferson.edu.

PMID: 37714216
DOI: 10.1016/j.jaad.2023.08.084

Abstract

In the second part of this Continuing Medical Education article on paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome (PNP/PAMS), its diagnostic criteria, investigative work-up, and management are reviewed. PNP/PAMS is a rare autoimmune blistering disorder associated with high morbidity and mortality. Recognizing PNP/PAMS's key features and its diagnostic criteria is critical in initiating appropriate work-up. Evaluating PNP/PAMS requires knowledge of its findings on histopathology, direct immunofluorescence, indirect immunofluorescence, and enzyme-linked immunosorbent assay. Lastly, treatments for PNP/PAMS are reviewed with suggestions based on case reports and expert opinions in the literature. LEARNING OBJECTIVES: After completing this learning objective, the reader will be able to identify the criteria necessary for diagnosing paraneoplastic pemphigus (PNP/PAMS), learn how to work-up a diagnosis of PNP/PAMS, and understand important principles in the management of PNP/PAMS.

Keywords: ELISA; PAMS; criteria; diagnosis; histopathology; paraneoplastic; paraneoplastic autoimmune syndrome; pemphigus; therapy; treatment.

Publication types

Review