大疱性类天疱疮合并原发性腹膜后弥漫大B细胞淋巴瘤1例

Bullous Pemphigoid with Diffuse Large B-cell Lymphoma Primarily Presented in the Retroperitoneum

患者男,77岁。躯干、四肢反复红斑、水疱1年余,加重10d,确诊为大疱性类天疱疮。患者发病4年后体检时发现腹膜后肿物,外科手术切除肿物后组织病理显示淋巴样细胞弥漫性浸润,免疫组化示CD20(+),PAX-5(+),Bcl-6(灶+),MUM-1(+),Ki-67(90%+)。诊断:原发性腹膜后弥漫大B细胞淋巴瘤。

A 77-year-old male presented with recurrent erythema, blisters and bullae on his body more than one year before, and was diagnosed with bullous pemphigoid （BP）. Four years later MRI examination showed a retroperitoneal mass. Histopathologic findings showed a dense diffuse infiltration of lymphatic cells. Immunohisto- chemical staining showed CD20, PAX-5, Bcl-6, MUM-I, Ki-67 were positive. The final diagnosis is diffuse large B-cell lymphoma primarily presenting in the retroperitoneum.