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副肿瘤性天疱疮(paraneoplastic pemphigus,PNP)是一种与副肿瘤性自身免疫相关的皮肤黏膜疾病[1]。PNP的发生多与血液系统恶性肿瘤或淋巴增生性疾病有关,其典型的临床症状包括顽固性口腔炎、多形性皮损,常伴有除皮肤外的全身多器官受累。笔者报道了1例与纵隔巨大胸腺瘤相关的PNP病例。
副肿瘤性天疱疮伴发纵隔巨大胸腺瘤18F-FDG PET/CT显像1例
Paraneoplastic pemphigus with mediastinal giant thymoma 18F-FDG PET/CT imaging: a case report
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摘要: 副肿瘤性天疱疮(PNP)是一种与肿瘤相关的皮肤黏膜自身免疫性疾病,其伴发纵隔巨大胸腺瘤在临床较罕见。笔者报道了1例PNP患者,从临床、组织病理学、治疗、18F-氟脱氧葡萄糖(FDG) PET/CT显像等方面分析了PNP的特点,通过文献复习加深了对该病的认识。多形性皮肤损害患者应考虑PNP的可能,其诊断基于临床表现、组织病理学检查以及免疫荧光检查。笔者通过PET/CT显像发现隐匿性的恶性肿瘤,辅助临床医师了解胸腺瘤与PNP的内在联系,提高术前的诊断准确率,并为治疗方案的选择提供重要依据。Abstract: Paraneoplastic pemphigus (PNP) is a tumor-related autoimmune disease affecting the skin and mucosa, while the occurrence of mediastinal giant thymoma with PNP is infrequent in clinical practice. In this study, the authors presents a case of PNP and conducts a comprehensive analysis of its clinical, histopathological, therapeutic, and 18F-fluorodeoxyglucose (FDG) PET/CT imaging characteristics, thereby deepening the understanding of this disease through literature review. The possibility of PNP should be considered in patients with polymorphic skin lesions, the diagnosis of which is based on clinical presentation, histopathologic examination, and immunofluorescence. The autors found occult malignant tumors by PET/CT imaging, which assisted clinicians to understand the intrinsic connection between thymoma and PNP, improved the diagnostic accuracy of preoperative diagnosis, and provided an important basis for the choice of treatment plan.
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[1] Kim JH, Kim SC. Paraneoplastic pemphigus: paraneoplastic autoimmune disease of the skin and mucosa[J/OL]. Front Immunol, 2019, 10: 1259[2022-06-07]. https://www.frontiersin.org/articles/10.3389/fimmu.2019.01259/full. DOI: 10.3389/fimmu.2019.01259. [2] Anhalt GJ, Kim SC, Stanley JR, et al. Paraneoplastic pemphigus-an autoimmune mucocutaneous disease associated with neoplasia[J]. N Engl J Med, 1990, 323(25): 1729−1735. DOI: 10.1056/NEJM199012203232503. [3] Tirado-Sánchez A, Bonifaz A. Paraneoplastic Pemphigus. A life-threatening autoimmune blistering disease[J]. Actas Dermosifiliogr, 2017, 108(10): 902−910. DOI: 10.1016/j.ad.2017.04.024. [4] Paolino G, Didona D, Magliulo G, et al. Paraneoplastic pemphigus: insight into the autoimmune pathogenesis, clinical features and therapy[J/OL]. Int J Mol Sci, 2017, 18(12): 2532[2022-06-07]. https://www.mdpi.com/1422-0067/18/12/2532. DOI: 10.3390/ijms18122532. [5] Liu Q, Bu DF, Li D, et al. Genotyping of HLA-Ⅰ and HLA-Ⅱ alleles in Chinese patients with paraneoplastic pemphigus[J]. Br J Dermatol, 2008, 158(3): 587−591. DOI: 10.1111/j.1365-2133.2007.08361.x. [6] Costan VV, Popa C, Hâncu MF, et al. Comprehensive review on the pathophysiology, clinical variants and management of pemphigus (Review)[J]. Exp Ther Med, 2021, 22(5): 1335. DOI: 10.3892/etm.2021.10770. [7] Amber KT, Valdebran M, Grando SA. Paraneoplastic autoimmune multiorgan syndrome (PAMS): beyond the single phenotype of paraneoplastic pemphigus[J]. Autoimmun Rev, 2018, 17(10): 1002−1010. DOI: 10.1016/j.autrev.2018.04.008. [8] Didona D, Fania L, Didona B, et al. Paraneoplastic dermatoses: a brief general review and an extensive analysis of paraneoplastic pemphigus and paraneoplastic dermatomyositis[J/OL]. Int J Mol Sci, 2020, 21(6): 2178[2022-06-07]. https://www.mdpi.com/1422-0067/21/6/2178. DOI: 10.3390/ijms21062178. [9] Svoboda SA, Huang SM, Liu XY, et al. Paraneoplastic pemphigus: revised diagnostic criteria based on literature analysis[J]. J Cutan Pathol, 2021, 48(9): 1133−1138. DOI: 10.1111/cup.14004. [10] Li YC, Zhang Y, Fang Q, et al. Radiomics analysis of [18F]FDG PET/CT for microvascular invasion and prognosis prediction in very-early- and early-stage hepatocellular carcinoma[J]. Eur J Nucl Med Mol Imaging, 2021, 48(8): 2599−2614. DOI: 10.1007/s00259-020-05119-9. [11] Khiewvan B, Ziai P, Houshmand S, et al. The role of PET/CT as a prognosticator and outcome predictor in lung cancer[J]. Expert Rev Respir Med, 2016, 10(3): 317−330. DOI: 10.1586/17476348.2016.1147959. [12] Seki N, Sakamoto S, Karube Y, et al. ¹8F-fluorodeoxyglucose positron emission tomography for evaluation of thymic epithelial tumors: utility for World Health Organization classification and predicting recurrence-free survival[J]. Ann Nucl Med, 2014, 28(3): 257−262. DOI: 10.1007/s12149-014-0804-2. [13] Frew JW, Murrell DF. Current management strategies in paraneoplastic pemphigus (paraneoplastic autoimmune multiorgan syndrome)[J]. Dermatol Clin, 2011, 29(4): 607−612. DOI: 10.1016/j.det.2011.06.016.