A Case Report of Paraneoplastic Pemphigus Associated With Retroperitoneal Inflammatory Myofibroblastic Tumor

摘要

Paraneoplastic pemphigus (PNP) is an autoimmune bullous disease associated with underlyingneoplasms, both malignant and benign. The most constant clinical presentation of PNP is the presence ofintractable stomatitis. Herein we present a 25-year-old male with a 3-month history of refractory stomatitisespecially involving the lips and widespread vesiculobullous eruption on his trunk and extremities. Thediagnosis of PNP was confirmed based on histological and serological results. Investigation for theunderlying neoplasm revealed a retroperitoneal tumorous mass which was biopsied and diagnosed as theinflammatory myofibroblastic tumor (IMT). The tumor was surgically excised, and different treatmentregimens were used to treat the mucocutaneous lesions. Skin lesions responded favorably to treatment, butoral stomatitis still persists which is the case in most PNP patients. This combination of PNP and IMT has