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线性硬皮病与原发性硬化性胆管炎：一种未被描述的关联。

Linear Scleroderma and Primary Sclerosing Cholangitis: An Undescribed Association.

作者信息

Fernández Iñigo Navarro, Alonso Susana Armesto, Vian Carlos Duran, Cura L Reguero Del, González-Vela Carmen, González-López Marcos

机构信息

Department of Dermatology, Hospital Universitario Marqués de Valdecilla, Santander, Spain.

Department of Pathology, Hospital Universitario Marqués de Valdecilla, Santander, Spain.

出版信息

Indian J Dermatol. 2020 Nov-Dec;65(6):514-515. doi: 10.4103/ijd.IJD_473_18.

DOI:10.4103/ijd.IJD_473_18
PMID:33487709
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7810082/
Abstract

Linear scleroderma (LS) is clinically characterized by the presence of sclerotic areas of skin, which develop in a linear pattern. Primary sclerosing cholangitis is a cholestatic disorder that can lead to end-stage liver disease. We present, for the first time in English literature, the case of a patient suffering from both the diseases. This highlights the fact that, even though LS has conventionally been considered to be a form of localized scleroderma, this does not necessarily imply that it is an exclusively cutaneous disease.

摘要

线性硬皮病（LS）的临床特征是皮肤出现硬化区域，呈线性发展。原发性硬化性胆管炎是一种胆汁淤积性疾病，可导致终末期肝病。我们首次在英文文献中报道了一名同时患有这两种疾病的患者。这凸显了一个事实，即尽管传统上认为LS是局限性硬皮病的一种形式，但这并不一定意味着它仅仅是一种皮肤疾病。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3211/7810082/72e9768c8200/IJD-65-514-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3211/7810082/72e9768c8200/IJD-65-514-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3211/7810082/72e9768c8200/IJD-65-514-g001.jpg

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本文引用的文献

1
Skin Manifestations Associated with Autoimmune Liver Diseases: a Systematic Review.自身免疫性肝病相关的皮肤表现：系统评价。
Clin Rev Allergy Immunol. 2017 Dec;53(3):394-412. doi: 10.1007/s12016-017-8649-9.
2
Scleroderma and liver disease: a case of an association with primary sclerosing cholangitis.硬皮病与肝脏疾病：一例与原发性硬化性胆管炎相关的病例。
Scand J Rheumatol. 2016 Jul;45(4):334-5. doi: 10.3109/03009742.2015.1114667. Epub 2015 Dec 21.
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Primary sclerosing cholangitis associated with CREST (calcinosis, Raynaud phenomenon, oesophageal dysmotility, sclerodactyly and telangiectasia) in an elderly woman: a case report.
一名老年女性中与CREST（钙质沉着、雷诺现象、食管动力障碍、指端硬化和毛细血管扩张）相关的原发性硬化性胆管炎：病例报告
J Med Case Rep. 2015 Nov 25;9:272. doi: 10.1186/s13256-015-0747-9.
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Pathogenesis of primary sclerosing cholangitis.原发性硬化性胆管炎的发病机制。
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Endothelial cell apoptosis is a primary pathogenetic event underlying skin lesions in avian and human scleroderma.内皮细胞凋亡是禽类和人类硬皮病皮肤病变潜在的主要致病事件。
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Primary sclerosing cholangitis associated with systemic sclerosis.原发性硬化性胆管炎伴系统性硬化症。
Postgrad Med J. 1991 Feb;67(784):189-92. doi: 10.1136/pgmj.67.784.189.