IR@PKUHSC  > 北京大学第一临床医学院  > 皮肤性病科
学科主题临床医学
Paraneoplastic plemphigus
Zhu, Xuejun; Zhang, Bingxin
关键词associated tumor autoantibody paraneoplastic pemphigus removal tumor
刊名JOURNAL OF DERMATOLOGY
2007-08-01
DOI10.1111/j.1346-8138.2007.00322.x
34期:8页:503-511
收录类别SCI
文章类型Review
WOS标题词Science & Technology
类目[WOS]Dermatology
研究领域[WOS]Dermatology
关键词[WOS]DENDRITIC CELL-SARCOMA ; AUTOIMMUNE MULTIORGAN SYNDROME ; CHRONIC LYMPHOCYTIC-LEUKEMIA ; PEMPHIGOIDES-LIKE ERUPTION ; EROSIVE LICHEN-PLANUS ; CASTLEMANS-DISEASE ; BRONCHIOLITIS OBLITERANS ; PULMONARY INVOLVEMENT ; RESPIRATORY-FAILURE ; BULLOUS DISEASE
英文摘要

Paraneoplastic pemphigus (PNP) is a life-threatening autoimmune blistering skin disease. Clinically, it is characterized by severe mucosal erosions and various cutaneous lesions associated with lymphoproliferative neoplasmas. Suprabasal acantholysis and clefts with scattered necrotic keratinocytes are the unique histopathological features. PNP patient sera recognize multiple antigens, which have been identified as the plakin protein family that includes desmoplakin, bullous pemphigoid antigen I (BPAG1), envoplakin and periplakin, and clesmogleins 1 and 3. Castleman′s tumor, non-Hodgkin′s lymphoma, thymoma, follicular dendritic cell sarcoma and chronic lymphocytic leukemia are the commonly associated neoplasmas in PNP. We have also demonstrated that the autoantibodies reacting to epidermal proteins are directly produced by the cells in the associated tumors. Bronchiolitis obliterans is frequently found in PNP and may cause respiratory failure and death. In our experience, the early detection and removal of the tumor and i.v. administration of immunoglobulin are critical for the treatment of PNR

语种英语
WOS记录号WOS:000249014900001
Citation statistics
Cited Times:66[WOS]   [WOS Record]     [Related Records in WOS]
文献类型期刊论文
条目标识符http://ir.bjmu.edu.cn/handle/400002259/59019
Collection北京大学第一临床医学院_皮肤性病科
作者单位Peking Univ, First Hosp, Dept Dermatol, Beijing 100034, Peoples R China
Recommended Citation
GB/T 7714
Zhu, Xuejun,Zhang, Bingxin. Paraneoplastic plemphigus[J]. JOURNAL OF DERMATOLOGY,2007,34(8):503-511.
APA Zhu, Xuejun,&Zhang, Bingxin.(2007).Paraneoplastic plemphigus.JOURNAL OF DERMATOLOGY,34(8),503-511.
MLA Zhu, Xuejun,et al."Paraneoplastic plemphigus".JOURNAL OF DERMATOLOGY 34.8(2007):503-511.
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