Imataki Osamu, Tamai Yotaro, Abe Yoshiaki, Ito Ichiro, Yoshikawa Shusuke, Kawakami Kimihiro
Division of Hematology and Stem Cell Transplantation, Shizuoka Cancer Center.
Gan To Kagaku Ryoho. 2006 Nov;33(11):1677-80.
Paraneoplastic pemphigus (PNP) is a rare autoimmune bullous disease associated with neoplasm, which is clinically characterized by mucocutaneous lesions resembling pemphigus vulgaris or erythema multiforme. A case presented with PNP refractory to chemotherapy including rituximab, predonisolone and cyclophosphamide (RCHOP regimen). A 36-year-old man, who had been diagnosed as extended follicular lymphoma, presented with a polymorphous skin eruption of the trunk, sclera conjunctivitis, and severe mucosal erosions of the lips and oral cavity. He was diagnosed as pemphigus pathologically by a biopsy of the oral mucosa. However, 3 courses of rituximab and CHOP therapy, which exert a partial response with lymphoma lesions, did not prove effective for oral stomatitis due to pemphigus. He received corticosteroid therapy (prednisolone 40 mg/day) and went into a state of temporally remission regarding pemphigus. However, the mucosal lesions were again exacerbated despite control of the lymphoma status after chemotherapy. Oral stomatitis extended to the upper respiratory system through the larynx and resulted in bronchiolitis obliterance clinically presented likely as severe chronic obstructive pulmonary disease (COPD). Because it is known that PNP refractory to long-term steroid and cytoreductive therapy has a progressive character and poor prognosis, supportive care would be warranted for these patients.
副肿瘤性天疱疮（PNP）是一种与肿瘤相关的罕见自身免疫性大疱性疾病，临床特征为类似寻常型天疱疮或多形红斑的黏膜皮肤损害。有一例对包括利妥昔单抗、泼尼松龙和环磷酰胺（RCHOP方案）在内的化疗难治的PNP病例。一名36岁男性，被诊断为弥漫性滤泡性淋巴瘤，出现躯干多形性皮疹、巩膜结膜炎以及唇部和口腔严重黏膜糜烂。通过口腔黏膜活检病理诊断为天疱疮。然而，3个疗程的利妥昔单抗和CHOP治疗对淋巴瘤病变有部分反应，但对天疱疮所致口腔炎无效。他接受了皮质类固醇治疗（泼尼松龙40mg/天），天疱疮暂时缓解。然而，尽管化疗后淋巴瘤病情得到控制，但黏膜损害再次加重。口腔炎通过喉部蔓延至上呼吸道，导致细支气管炎，临床上可能表现为严重的慢性阻塞性肺疾病（COPD）。由于已知对长期类固醇和细胞减灭治疗难治的PNP具有进行性特点且预后不良，因此应对这些患者进行支持治疗。
