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腹膜后CASTLEMAN病伴副肿瘤性天疱疮1例报道并文献复习

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目的 探讨腹膜后CASTLEMAN病(RPCD)伴副肿瘤性天疱疮(PNP)的特点及诊治经验.方法 回顾性分析我院诊治的1例RPCD伴PNP病人的临床资料,并查阅相关文献进行复习.结果 病人为33岁男性,以口腔天疱疮为首发症状,糖皮质激素治疗效果不佳,口腔黏膜活检病理检查示天疱疮.盆腔增强CT检查提示腹膜后占位性病变,行开腹手术,术后病理检查提示RPCD,术后天疱疮症状逐渐减轻.结论 该病罕见,其诊断常被延误,手术是治疗该病的根本方法,但术后皮肤黏膜症状可持续较长时间.
ONE CASE OF RETROPERITONEAL CASTLEMAN DISEASE COMPLICATED BY PARANEOPLASTIC PEMPHIGUS AND LITERATURE REVIEW
Objective To investigate the features of retroperitoneal CASTLEMAN disease (RPCD) complicated by paraneoplastic pemphigus (PNP) and related diagnosis and treatment experience.Methods A retrospective analysis was performed for the clinical data of one patient with RPCD complicated by PNP who were diagnosed and treated in our hospital,and literature review was also performed.Results The patient was a 33-year-old man with an initial symptom of oral pemphigus and had poor response to glucocorticoids.Oral mucosal biopsy showed pemphigus.Pelvic contrast-enhanced computed tomography showed a retroperitoneal space-occupying lesion.An open surgery was performed and postoperative pathological examination suggested RPCD.The symptom of pemphigus was gradually alleviated after surgery:Conclusion This disease is rare and its diagnosis is often delayed.Surgery is the radical treatment of this disease,but skin and mucosal symptoms may last for a long time after surgery.

retroperitoneal neoplasmsGiant Lymph Node Hyperplasiaparaneoplastic syndromesdiagnosis

曲腾飞、宋晓峰、马广涛、于才艳、邱法波

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青岛大学附属医院肝胆胰腺外科,山东青岛266003

青岛大学附属医院急诊普外科,山东青岛266003

腹膜后肿瘤 巨淋巴结增生 副肿瘤综合征 诊断

2017

齐鲁医学杂志
青岛大学医学院

齐鲁医学杂志

影响因子:0.609
ISSN:1008-0341
年,卷(期):2017.32(3)
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