Clinical Characterization of Oral Symptoms in 6 Paraneoplastic Pemphigus Patients

  • FUJITA Kohei
    Department of Dentistry and Oral Surgery, Keio University School of Medicine
  • SATO Hidekazu
    Department of Dentistry and Oral Surgery, Keio University School of Medicine
  • KATO Shin
    Department of Dentistry and Oral Surgery, Keio University School of Medicine
  • SAISU Hironori
    Department of Dentistry and Oral Surgery, Keio University School of Medicine Multidisciplinary Pain Center, Aichi Medical University
  • IKEURA Kazuhiro
    Department of Dentistry and Oral Surgery, Keio University School of Medicine
  • KODAKA Rie
    Department of Dentistry and Oral Surgery, Keio University School of Medicine
  • TSUNODA Hiroyuki
    Department of Dentistry and Oral Surgery, Keio University School of Medicine
  • MORI Takehiko
    Division of Hematology, Department of Internal Medicine, Keio University School of Medicine
  • YAMAGAMI Jun
    Department of Dermatology, Keio University School of Medicine
  • AMAGAI Masayuki
    Department of Dermatology, Keio University School of Medicine
  • NAKAGAWA Taneaki
    Department of Dentistry and Oral Surgery, Keio University School of Medicine
  • TSUNODA Kazuyuki
    Department of Dentistry and Oral Surgery, Keio University School of Medicine

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Other Title
  • 腫瘍随伴性天疱瘡6例の口腔症状に関する臨床的検討
  • シュヨウ ズイハンセイ テンポウソウ 6レイ ノ コウコウ ショウジョウ ニ カンスル リンショウテキ ケントウ

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Abstract

Paraneoplastic pemphigus(PNP)is a fetal autoimmune blistering disease mainly accompanied by a hematologic tumor manifesting various severe mucocutaneous lesions. To clarify the oral symptoms of PNP, we clinically analyzed six cases of PNP. The accompanying tumor was follicular lymphoma or thymoma. As typical oral symptoms, severe erosion, ulcer and lichenoid mucosal eruption in the oral mucosa and crust, blood crust and erosion of the lip were confirmed as the first manifestations of PNP. In addition, histopathologically, suprabasilar acantholysis and interface dermatitis caused by inflammatory cell infiltration were found. These clinical and histopathological characteristics were correlated with the autoimmune mechanism of both sides due to humoral and cell-mediated immunity. Given that oral symptoms are the first to occur, PNP patients are likely to visit a dentist for relief; as such, dentists should understand the pathophysiology, clinical manifestations and histopathological findings of PNP in order to make an accurate diagnosis in their dental practice.

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