Paraneoplastic pemphigus without antibodies to desmoglein 1 and 3

Kelsey Flood*, Christopher Stroud, Zelmira Lazarova, Nadarajah Vigneswaran, Sylvia Hsu

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

3 Scopus citations

Abstract

Paraneoplastic pemphigus is a severe autoimmune blistering disease presenting in the setting of underlying malignancy. Paraneoplastic pemphigus is associated with diffuse painful stomatitis throughout the oral cavity with extension to the lips. The cutaneous findings are varied and have been described as lichenoid, pemphigoid, and targetoid lesions. Herein, we report a patient with paraneoplastic pemphigus whose routine testing led to a diagnosis of pemphigus vulgaris. However, further testing was pursued revealing an antibody profile consistent with paraneoplastic pemphigus. Subsequent neoplastic workup revealed an intra-abdominal mass. Our case represents a subtle, non-classic presentation of paraneoplastic pemphigus and suggests the importance of a comprehensive investigative work-up in atypical cases of pemphigus.

Original languageEnglish (US)
Article number4
JournalDermatology online journal
Volume24
Issue number2
StatePublished - 2018

Keywords

  • Desmoglein
  • Desmoglein compensation theory
  • Paraneoplastic pemphigus
  • Stomatitis

ASJC Scopus subject areas

  • Dermatology

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