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

ELISA PAMS criteria diagnosis histopathology paraneoplastic paraneoplastic autoimmune syndrome pemphigus therapy treatment

Journal

Journal of the American Academy of Dermatology
ISSN: 1097-6787
Titre abrégé: J Am Acad Dermatol
Pays: United States
ID NLM: 7907132

Informations de publication

Date de publication:
13 Sep 2023
Historique:
received: 24 03 2023
revised: 09 08 2023
accepted: 11 08 2023
medline: 16 9 2023
pubmed: 16 9 2023
entrez: 15 9 2023
Statut: aheadofprint

Résumé

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.

Identifiants

pubmed: 37714216
pii: S0190-9622(23)02685-3
doi: 10.1016/j.jaad.2023.08.084
pii:
doi:

Types de publication

Journal Article Review

Langues

eng

Sous-ensembles de citation

IM

Informations de copyright

Copyright © 2023. Published by Elsevier Inc.

Auteurs

Simo Huang (S)

Department of Dermatology, Thomas Jefferson University Hospitals, Philadelphia, Pennsylvania.

Hannah J Anderson (HJ)

Department of Dermatology, Thomas Jefferson University Hospitals, Philadelphia, Pennsylvania.

Jason B Lee (JB)

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

Classifications MeSH