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IR@PKUHSC  > 北京大学第一临床医学院  > 皮肤性病科  > 期刊论文
学科主题: 临床医学
题名:
Paraneoplastic plemphigus
作者: Zhu, Xuejun; Zhang, Bingxin
关键词: associated tumor ; autoantibody ; paraneoplastic pemphigus ; removal tumor
刊名: JOURNAL OF DERMATOLOGY
发表日期: 2007-08-01
DOI: 10.1111/j.1346-8138.2007.00322.x
卷: 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:
内容类型: 期刊论文
URI标识: http://ir.bjmu.edu.cn/handle/400002259/59019
Appears in Collections:北京大学第一临床医学院_皮肤性病科_期刊论文

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作者单位: Peking Univ, First Hosp, Dept Dermatol, Beijing 100034, Peoples R China

Recommended Citation:
Zhu, Xuejun,Zhang, Bingxin. Paraneoplastic plemphigus[J]. JOURNAL OF DERMATOLOGY,2007,34(8):503-511.
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