Paraneoplastic pemphigus: Insight into the autoimmune pathogenesis, clinical features and therapy

Giovanni Paolino, Dario Didona, Giuseppe Magliulo, Giannicola Iannella, Biagio Didona, Santo Raffaele Mercuri, Elisa Moliterni, Michele Donati, Andrea Ciofalo, Guido Granata, Patricia Ranuzzi, Vincenzo Falasca, Stefano Calvieri

Research output: Contribution to journalReview articlepeer-review


Paraneoplastic pemphigus is a rare autoimmune skin disease that is always associated with a neoplasm. Usually, oral, skin, and mucosal lesions are the earliest manifestations shown by paraneoplastic pemphigus patients. The pathogenesis of paraneoplastic pemphigus is not yet completely understood, although some immunological aspects have been recently clarified. Because of its rarity, several diagnostic criteria have been proposed. Besides, several diagnostic procedures have been used for the diagnosis, including indirect immunofluorescence, direct immunofluorescence, and ELISA. We reviewed the most recent literature, searching on PubMed “paraneoplastic pemphigus”. We included also papers in French, German, and Spanish. We found 613 papers for “paraneoplastic pemphigus”. Among them, 169 were review papers. Because of its varying clinical features, paraneoplastic pemphigus still represents a challenge for clinicians. Furthermore, diagnosis and management of paraneoplastic pemphigus requires close collaboration between physicians, including dermatologist, oncologist, and otorhinolaryngologist.

Original languageEnglish
Article number2532
JournalInternational Journal of Molecular Sciences
Issue number12
Publication statusPublished - Dec 1 2017


  • Oncology
  • Oral lesions
  • Paraneoplastic pemphigus
  • Therapy

ASJC Scopus subject areas

  • Catalysis
  • Molecular Biology
  • Spectroscopy
  • Computer Science Applications
  • Physical and Theoretical Chemistry
  • Organic Chemistry
  • Inorganic Chemistry


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