A case of paraneoplastic pemphigus without an underlying neoplasm initially associated with oral symptoms

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  • KITAMURA Naoya
    Department of Oral and Maxillofacial Surgery, Kochi Medical School, Kochi University
  • OHNO Seiji
    Department of Oral and Maxillofacial Surgery, Kochi Medical School, Kochi University
  • YOSHIMURA Tomohide
    Department of Oral and Maxillofacial Surgery, Kochi Medical School, Kochi University
  • SASABE Eri
    Department of Oral and Maxillofacial Surgery, Kochi Medical School, Kochi University
  • YAMADA Tomohiro
    Department of Oral and Maxillofacial Surgery, Kochi Medical School, Kochi University
  • YAMAMOTO Tetsuya
    Department of Oral and Maxillofacial Surgery, Kochi Medical School, Kochi University

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Other Title
  • 口腔内症状を初発とし随伴腫瘍が同定されなかった腫瘍随伴性天疱瘡の1例
  • コウコウ ナイ ショウジョウ オ ショハツ ト シ ズイハン シュヨウ ガ ドウテイ サレナカッタ シュヨウ ズイハンセイ テンポウソウ ノ 1レイ

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Paraneoplastic pemphigus is an exclusive subset of pemphigus initially reported by Anhalt et al. in 1990. We report a case of paraneoplastic pemphigus initially presenting with oral mucosal erosions and ulcers without an underlying neoplasm that was diagnosed on the basis of clinical, pathological, and immunological findings. The patient was a 67-year-old woman with erosions and ulcers of buccal mucosa, tongue, and lips. Either multiform exudative erythema or pemphigus was suspected, and topical steroid ointment and oral prednisolone were administered. However, oral symptoms did not improve. In addition to the exacerbation of oral mucosal lesions, pale skin erythema appeared on the trunk of the body. Since Stevens-Johnson syndrome was suspected by a dermatologist in our hospital, steroid pulse therapy was administered, followed by chemotherapy with oral prednisolone and cyclosporine A; however, oral mucosal symptoms worsened. Direct immunofluorescence studies showed the deposition of complement in the epidermal basement zone, and indirect immunofluorescence studies indicated the deposition of immunoglobulin G on stained sections of monkey esophagus and rat urinary bladder. Furthermore, immunoblot studies using human kerationocyte extracts revealed autoantibodies against 190kDa (periplakin) and 210kDa (envoplakin) proteins in the patient's serum. Although the patient was finally given a diagnosis of paraneoplastic pemphigus on the basis of these immunological features, no underlying neoplasm could be identified. Oral mucosa and skin symptoms were improved by plasma exchange therapy. However, the patient died of bronchiolitis obliterans approximately 9 months after the initial visit.

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