Paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome: Part I. clinical overview and pathophysiology.
CLL
Castleman disease
bronchiolitis obliterans
non-Hodgkin lymphoma
paraneoplastic
paraneoplastic autoimmune multiorgan syndrome
paraneoplastic pemphigus
pemphigus
plakin
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:
17 Aug 2023
17 Aug 2023
Historique:
received:
24
03
2023
revised:
24
07
2023
accepted:
07
08
2023
pubmed:
20
8
2023
medline:
20
8
2023
entrez:
19
8
2023
Statut:
aheadofprint
Résumé
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.
Identifiants
pubmed: 37597771
pii: S0190-9622(23)02512-4
doi: 10.1016/j.jaad.2023.08.020
pii:
doi:
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Informations de copyright
Copyright © 2023 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.
Déclaration de conflit d'intérêts
Conflicts of interest None disclosed.