口腔に発生した腫瘍随伴性天疱瘡の1例

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  • A case of paraneoplastic pemphigus arising in the oral cavity
  • 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

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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.

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