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嗜铬细胞瘤患者并发副肿瘤性天疱疮:1例罕见病例报告

Paraneoplastic Pemphigus in a Patient With Pheochromocytoma: A Report of a Rare Case.

作者信息

Huang Zhong-Zhou, He Ming-Jie, Huang Ping, Luo Min-Qing, Hong Dan, Lu Sha, Guo Qing, Wang Liangchun, Xiong Hui

机构信息

Department of Dermatology, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, CHN.

Department of Epidemiology and Public Health, School of Public Health, Sun Yat-sen University, Guangzhou, CHN.

出版信息

Cureus. 2025 Feb 22;17(2):e79459. doi: 10.7759/cureus.79459. eCollection 2025 Feb.

Abstract

This report details a rare case of paraneoplastic pemphigus (PNP) associated with pheochromocytoma. The patient presented with prominent dermatological manifestations, including erythema, vesicles, and erosions. During hospitalization, diagnostic imaging revealed a retroperitoneal mass, which was subsequently surgically removed. A comprehensive diagnostic workup, including CT, MRI, histopathological analysis, and direct immunofluorescence examination, was conducted. Postoperative management combined with pharmacological intervention led to significant clinical improvement. This case highlights the critical importance of considering PNP in the differential diagnosis of pheochromocytoma, particularly in patients presenting with complex autoimmune manifestations. The findings underscore the necessity for early diagnosis and prompt therapeutic intervention in such cases. Additionally, this report emphasizes the need for further investigation into the clinical spectrum, genetic associations, and underlying mechanisms of this rare disease association to enhance diagnostic accuracy and therapeutic outcomes.

摘要

本报告详细介绍了一例与嗜铬细胞瘤相关的副肿瘤性天疱疮(PNP)罕见病例。患者表现出明显的皮肤病学表现,包括红斑、水疱和糜烂。住院期间,诊断性影像学检查发现腹膜后肿块,随后进行了手术切除。进行了全面的诊断检查,包括CT、MRI、组织病理学分析和直接免疫荧光检查。术后管理结合药物干预导致临床显著改善。该病例突出了在嗜铬细胞瘤的鉴别诊断中考虑PNP的至关重要性,特别是在出现复杂自身免疫表现的患者中。这些发现强调了在此类病例中早期诊断和及时治疗干预的必要性。此外,本报告强调需要进一步研究这种罕见疾病关联的临床谱、基因关联和潜在机制,以提高诊断准确性和治疗效果。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/769a/11933724/859d4faead36/cureus-0017-00000079459-i01.jpg

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