Paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome: Part I. Clinical overview and pathophysiology.
Publication/Presentation Date
7-1-2024
Abstract
Paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome (PNP/PAMS) is a highly fatal autoimmune blistering disease. The condition occurs in patients with underlying benign or malignant neoplasms, most commonly lymphoproliferative disorders. Both humoral and cell-mediated immunities contribute to the pathogenesis, and autoantibodies against plakin family proteins are characteristic. Patients typically present with severe stomatitis and polymorphous skin lesions, which are often resistant to treatment. Bronchiolitis obliterans (BO) is a frequent complication which contributes to the high mortality rate of PNP/PAMS. Given the rarity of this disorder and heterogeneity of clinical presentation, clinicians should maintain a high index of suspicion for PNP/PAMS to avoid delayed diagnosis. In this first part of a two-part continuing medical education (CME) series, risk factors, pathogenesis, and clinical features of PNP/PAMS are discussed.
Volume
91
Issue
1
First Page
1
Last Page
10
ISSN
1097-6787
Published In/Presented At
Anderson, H. J., Huang, S., & Lee, J. B. (2024). Paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome: Part I. Clinical overview and pathophysiology. Journal of the American Academy of Dermatology, 91(1), 1–10. https://doi.org/10.1016/j.jaad.2023.08.020
Disciplines
Medicine and Health Sciences
PubMedID
37597771
Department(s)
Department of Pathology and Laboratory Medicine
Document Type
Article