报告1例盆腔Castleman病并发副肿瘤性天疱疮。患儿男,13岁。口腔黏膜糜烂3个月余。皮肤科检查:患儿双侧眼角、口唇、口腔黏膜和舌面糜烂,覆血痂。双手指甲变形、剥离,甲皱襞红斑,龟头、冠状沟、肛门红肿糜烂。右下腹麦氏点左下方23 cm处触一肿物。全身PET/CT:盆腔内巨大不规则肿块,伴钙化。术后皮损组织病理检查:盆腔血管滤泡性淋巴结增生,透明血管型。诊断:盆腔Castleman病并发副肿瘤性天疱疮。 One case of pelvic Castleman’s disease complicated with paraneoplastic pemphigus was reported. Children male, 13 years old. Oral mucosal erosion more than 3 months. Dermatology examination: children with bilateral eyes, lips, oral mucosa and tongue erosion, blood clots. Nail deformity, stripping, nail folds erythema, glans, coronal, anal swelling and erosion. Right lower quadrant of the lower left 23 cm at a point touching the tumor. Whole body PET / CT: pelvic huge irregular mass, with calcification. Postoperative histopathological examination of skin lesions: pelvic vascular follicular lymph node hyperplasia, transparent vascular type. Diagnosis: Pelvic Castleman’s disease complicated with paraneoplastic pemphigus.