伴恶性胸腺瘤和闭塞性细支气管炎的副肿瘤性天疱疮一例

王瑞; 王明悦

doi:10.12376/j.issn.2097-0501.2023.02.011

伴恶性胸腺瘤和闭塞性细支气管炎的副肿瘤性天疱疮一例

doi: 10.12376/j.issn.2097-0501.2023.02.011

王瑞,
王明悦^,

北京大学第一医院皮肤性病科国家皮肤与免疫疾病临床医学研究中心国家药品监督管理局化妆品质量控制与评价重点实验室北京市皮肤病分子诊断重点实验室，北京 100034

详细信息

通信作者:
王明悦，E-mail：wangmy@pku.edu.cn

中图分类号: R445;R751
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出版历程
- 收稿日期: 2022-12-27
- 录用日期: 2023-01-12
- 网络出版日期: 2023-05-05

A Case of Paraneoplastic Pemphigus with Malignant Thymoma and Obliterated Bronchiolitis

WANG Rui,
WANG Mingyue^,

Department of Dermatology, Peking University First Hospital & National Clinical Research Center for Skin and Immune Diseases & NMPA Key Laboratory for Quality Control and Evaluation of Cosmetics & Beijing Key Laboratory of Molecular Diagnosis on Dermatoses, Beijing 100034, China

More Information

Corresponding author: WANG Mingyue, E-mail: wangmy@pku.edu.cn

摘要

摘要: 副肿瘤性天疱疮(PNP)是一种罕见的、伴发淋巴细胞增生性肿瘤的危重症自身免疫性大疱性疾病，临床表现为严重的口腔黏膜糜烂及多形性的皮肤损害。Castleman病、非霍奇金淋巴瘤、胸腺瘤、滤泡树突状肉瘤及慢性淋巴细胞性白血病是PNP常见的伴发肿瘤，然而伴发恶性胸腺瘤则较为罕见。本病预后极差，死亡率高，死亡原因通常包括严重的感染、潜在的恶性肿瘤及闭塞性细支气管炎。本文报道1例58岁女性反复口腔糜烂溃疡3年，全身红斑水疱10个月，呼吸困难3个月，经临床、影像学、组织病理学、免疫学检查最终诊断为伴恶性胸腺瘤及闭塞性细支气管炎的PNP，该患者在恶性胸腺瘤切除后3周死于呼吸衰竭。
- 副肿瘤性天疱疮 /
- 闭塞性细支气管炎 /
- 恶性胸腺瘤
Abstract: Paraneoplastic pemphigus (PNP) is a rare and life-threatening autoimmune blistering disease characterized by severe mucosal erosions and polymorphous cutaneous eruptions associated with lymphoproliferative neoplasmas. Castleman's disease, non-Hodgkin's lymphoma, thymoma, follicular dendritic cell sarcoma and chronic lymphocytic leukemia are the commonly associated neoplasmas in PNP, whereas malignant thymoma is rare. The prognosis of PNP is poor due to severe infections during immunosuppressive treatment, underlying malignancies and bronchiolitis obliterans mediated by autoimmunity. A 58-year-old woman who experienced recurrent oral erosive ulcer for 3 years, systemic erythema for 10 months, and dyspnea for 3 months was diagnosed with PNP with malignant thymoma and obliterated bronchiolitis by clinical, radiological, histopathological, and immunological examinations. She died of respiratory failure 3 weeks after resection of the malignant thymoma.
- paraneoplastic pemphigus /
- obliterative bronchiolitis /
- malignant thymoma
注释:

作者贡献：王瑞负责收集病例资料、文章撰写；王明悦负责临床诊治、审阅修改。

利益冲突：所有作者均声明不存在利益冲突。

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图 1 患者皮肤表现及纵隔肿物

A.头面毛发脱落，面部暗红斑、色沉；B.唇部糜烂、血痂，舌体糜烂；C.双手指甲板远端增厚、粗糙、色素改变，近端萎缩；D.腰部红斑水疱；E.右小腿及足背暗红斑基础上多发松弛性水疱伴结痂；F.术中肿物切除标本(大小约8 cm×8 cm)

Figure 1. Skin manifestation and mediastinal mass of the patient

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图 2 患者胸部X线及CT检查

A.正位胸片示右肺门纵隔影增宽，呈半球形凸入肺野；B.胸片侧位观；C.胸部CT横切面示前纵隔肿物；D.胸部CT矢状位纵隔窗示前纵隔肿物；E.胸部呼气相末CT肺窗示支气管壁增厚，管径增大；F.胸部CT肺窗示弥漫磨玻璃影和支气管扩张

Figure 2. X-ray and CT examination on the patient's chest

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图 3 患者间接免疫荧光、肿物组织病理及免疫组织化学染色

A.以鼠膀胱移行上皮为底物行间接免疫荧光示IgG沿表皮棘细胞间沉积；B.术后肿物行组织病理检查示侵袭性胸腺瘤，混合细胞型(HE×200)；C.肿物组织行免疫组化示CD3阳性(×400)；D.肿物组织行免疫组化示AE1/AE3强阳性(×400)

Figure 3. Indirect immunofluorescence, histological and immunohistochemical staining results of the patient's tumor

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参考文献(11)

[1]	Anhalt GJ, Kim SC, Stanley JR, et al. Paraneoplastic pemphigus. An autoimmune mucocutaneous disease associa-ted with neoplasia[J]. N Engl J Med, 1990, 323(25): 1729-1735. doi: 10.1056/NEJM199012203232503
[2]	Baum S, Sakka N, Artsi O, et al. Diagnosis and classification of autoimmune blistering diseases[J]. Autoimmun Rev, 2014, 13(4-5): 482-489. doi: 10.1016/j.autrev.2014.01.047
[3]	Lee SE, Hashimoto T, Kim SC. No mucosal involvement in a patient with paraneoplastic pemphigus associated with thymoma and myasthenia gravis[J]. Br J Dermatol, 2008, 159(4): 986-988. doi: 10.1111/j.1365-2133.2008.08763.x
[4]	Joly P, Richard C, Gilbert D, et al. Sensitivity and specificity of clinical, histologic, and immunologic features in the diagnosis of paraneoplastic pemphigus[J]. J Am Acad Dermatol, 2000, 43(4): 619-626. doi: 10.1067/mjd.2000.107488
[5]	Detterbeck FC, Zeeshan A. Thymoma: current diagnosis and treatment[J]. Chin Med J (Engl), 2013, 126(11): 2186-2191.
[6]	Choi Y, Nam KH, Lee JB, et al. Retrospective analysis of 12 Korean patients with paraneoplastic pemphigus[J]. J Dermatol, 2012, 39(12): 973-981. doi: 10.1111/j.1346-8138.2012.01655.x
[7]	Kim A, Choi SJ, Kang CH, et al. Risk factors for developing post-thymectomy myasthenia gravis in patients with thymoma[J]. Muscle Nerve, 2021, 63(4): 531-537. doi: 10.1002/mus.27169
[8]	Lee J, Bloom R, Amber KT. A systematic review of patients with mucocutaneous and respiratory complications in paraneoplastic autoimmune multiorgan syndrome: Castleman's disease is the predominant malignancy[J]. Lung, 2015, 193(4): 593-596. doi: 10.1007/s00408-015-9732-8
[9]	Ouedraogo E, Gottlieb J, de Masson A, et al. Risk factors for death and survival in paraneoplastic pemphigus associated with hematologic malignancies in adults[J]. J Am Acad Dermatol, 2019, 80(6): 1544-1549. doi: 10.1016/j.jaad.2018.03.043
[10]	王仁贵, 李楠, 朱学俊, 等. Castleman病所致的与副肿瘤性天疱疮相关的闭塞性细支气管炎的临床、病理与影像[J]. 中华放射学杂志, 2003, 37(1): 63-67.
[11]	Wang M, Li F, Wang X, et al. Features and risk factors for paraneoplastic autoimmune multiorgan syndrome in 145 chinese patients[J]. Acta Derm Venereol, 2020, 100(18): adv00312. doi: 10.2340/00015555-3673