A case of paraneoplastic pemphigus arising in the oral cavity

• TSUNODA Kazuyuki

  Department of Dentistry and Oral Surgery, Keio University School of Medicine

• MITOMO Keisuke

  Department of Dentistry and Oral Surgery, Keio University School of Medicine

• SATO Hidekazu

  Department of Dentistry and Oral Surgery, Keio University School of Medicine

• YAMAGAMI Jun

  Department of Dermatology, Keio University School of Medicine

• NAKAGAWA Taneaki

  Department of Dentistry and Oral Surgery, Keio University School of Medicine

• KAWANA Hiromasa

  Department of Dentistry and Oral Surgery, Keio University School of Medicine

Bibliographic Information

Other Title

• 口腔に発生した腫瘍随伴性天疱瘡の1例
• コウコウ ニ ハッセイ シタ シュヨウ ズイハンセイ テンポウソウ ノ 1レイ

Abstract

Paraneoplastic pemphigus （PNP） is an autoimmune mucocutaneous blistering disease that occurs in association with underlying neoplasms. We report a case of orally-initiated PNP associated with thymoma. A 51-year-old man presented with erosive stomatitis affecting the floor of the mouth, buccal region, and soft palate mucosa. These lesions rapidly deteriorated, and Nikolsky phenomena with lichenoid changes was noted. A histological examination showed suprabasilar acantholysis with cell infiltration in the lamina propria. Direct immunofluorescence showed positivity for IgG and C3 in the intercellular spaces of the squamous epithelia. Indirect immunofluorescence showed IgG bound to the epithelium of the rat bladder. In addition, the patient serum reacted against 210 and 190 kDa proteins on immunoblotting. On computed tomography, a thymoma was detected in the anterior mediastinum. The patient showed severe oral blistering associated with thymoma and was finally given a diagnosis of PNP. Despite surgical excision of the thymoma, the oral symptoms persisted. To achieve remission, steroid pulse therapy, intravenous immunoglobulin therapy, cyclosporine treatment and plasma apheresis were additionally performed. We have been carefully following the activity of the oral symptoms and thymoma for 12 years. The characteristics leading to early diagnosis, including the clinical symptoms, serological test results, and diagnostic criteria of PNP, are discussed.

Journal

• Japanese Journal of Oral and Maxillofacial Surgery

  Japanese Journal of Oral and Maxillofacial Surgery 60 (5), 272-276, 2014

  Japanese Society of Oral and Maxillofacial Surgeons

Keywords

• paraneoplastic pemphigus
• thymoma
• oral symptoms

Details

• CRID: 1390001206532364160
• NII Article ID: 130005074258
• NII Book ID: AN00189163
• DOI: 10.5794/jjoms.60.272
• ISSN: 21861579
• ISSN: 00215163
• NDL BIB ID: 025552115
• Web Site: http://id.ndl.go.jp/bib/025552115
• Web Site: https://ndlsearch.ndl.go.jp/books/R000000004-I025552115
• Web Site: https://search.jamas.or.jp/link/ui/2014291248
• Text Lang: ja
• Data Source:
  • JaLC
  • NDL
  • Crossref
  • CiNii Articles
  • KAKEN
• Abstract License Flag: Disallowed