末梢性T細胞リンパ腫の化学療法中に生じた腫瘍随伴性天疱瘡の1例

抄録

Paraneoplastic pemphigus is a subtype of pemphigus that accompanies benign or malignant neoplasms. It is primarily accompanied by lymphoproliferative disorders, and manifests as painful oral mucositis along with a variety of skin lesions. Herein, we report the case of a 74-year-old woman with peripheral T-cell lymphoma who developed paraneoplastic pemphigus while on chemotherapy. During chemotherapy, the patient developed severe oral mucositis and was referred to our department because of the absence of improvement despite discontinuing chemotherapy 1 month earlier. Suspecting chemotherapy as the causative factor, we administered the patient with oral care; however, the oral mucositis worsened and bullous lesions developed on her extremities. We next suspected the cause to be paraneoplastic pemphigus and performed biopsies from an ulcer on the vermilion of the lip and from a bullous lesion on her skin. We treated her with oral prednisolone. Histopathological examination of the skin lesions revealed acanthosis, and an indirect immunofluorescence study revealed the intercellular deposition of immunoglobulin G in the transitional epithelium of the rat urinary bladder. Immunoblot analysis detected both envoplakin and periplakin proteins. Based on the results of our pathological analysis, paraneoplastic pemphigus was diagnosed. Her skin lesions improved noticeably; the oral lesions, while also showing some improvement, partially remained. Hence, the chemotherapy was restarted. However, the peripheral T-cell lymphoma worsened and she died of an underlying malignancy 6 months after her initial visit to our department.