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

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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.
日本口腔内科学会の論文