A Case of Paraneoplastic Pemphigus Initially Found as Extensive Ulceration of the Oral Mucosa

DOI 4 Citations 15 References Open Access
  • NAKANISHI Hiroaki
    First Department of Oral and Maxillofacial Surgery, School of Dentistry, University of Tokushima
  • SUMITOMO Takashi
    First Department of Oral and Maxillofacial Surgery, School of Dentistry, University of Tokushima
  • TOKUYAMA Reiko
    First Department of Oral and Maxillofacial Surgery, School of Dentistry, University of Tokushima
  • SATOMURA Kazuhito
    First Department of Oral and Maxillofacial Surgery, School of Dentistry, University of Tokushima
  • KAMATA Nobuyuki
    First Department of Oral and Maxillofacial Surgery, School of Dentistry, University of Tokushima
  • NAGAYAMA Masaru
    First Department of Oral and Maxillofacial Surgery, School of Dentistry, University of Tokushima
  • KANAGAWA Yasuhiko
    First Department of Internal Medicine, School of Medicine, University of Tokushima
  • MINAMI Mitsuyoshi
    Department of Dermatology, School of Medicine, University of Tokushima
  • HASHIMOTO Takashi
    Department of Dermatology, School of Medicine, Kurume University

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Other Title
  • 広範な口腔粘膜病変を初発症状とした腫瘍随伴性天疱瘡の1例

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A 49 year-old man presented with extensive ulceration and erosion in the mouth, followed by ocular erosion, bullus formation and erythema on the trunk, and penile ulcers. A CT examination showed a solid tumor in the posterior peritoneum, which revealed B cell-type malignant lymphoma. On serological examination, direct immunofluorescence testing showed IgG and C3 deposition in the epidermal intercellular spaces and basement membrane zone. Indirect immunofluorescence testing showed IgG deposition in the normal human epidermal intercellular spaces, and in the transitional epithelium of rat bladder. Immunoblot analysis demonstrated that the patient's serum reacted to envoplakin (210kDa) and periplakin (190kDa). According to these findings, we diagnosed this case as having PNP. Although a systemic steroid was administered for the mucosal-cutaneous lesions, and chemotherapy (CHOP) was conducted for the lymphoma, no remarkable subsidence was observed in the oral lesions. The patient died 2.5 months after the initial presentation.

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