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伴不明原因全血细胞减少的大疱性坏疽性脓皮病。

Bullous pyoderma gangrenosum associated with pancytopenia of unknown origin.

作者信息

Wolska Katarzyna, Michalska-Jakubus Małgorzata, Pucuła Joanna, Chodorowska Grażyna, Dzida Grzegorz, Mosiewicz Jerzy, Prystupa Andrzej, Podhorecka Monika, Krasowska Dorota

机构信息

Medical Students' Research Association at Department of Dermatology, Venereology and Paediatric Dermatology, Medical University of Lublin, Poland. Scientific supervisor: Prof. Dorota Krasowska MD, PhD.

Department of Dermatology, Venereology and Paediatric Dermatology, Medical University of Lublin, Poland. Head of Department: Prof. Grażyna Chodorowska MD, PhD.

出版信息

Postepy Dermatol Alergol. 2014 Aug;31(4):272-6. doi: 10.5114/pdia.2014.40980. Epub 2014 Sep 8.

Abstract

Pyoderma gangrenosum (PG) is a neutrophilic dermatosis of unknown origin. Clinically it starts with a pustule, nodule or bulla that rapidly progresses and turns into a painful ulcer with raised, undermined borders. The etiopathogenesis of PG remains unknown. However it is frequently associated with systemic diseases such as inflammatory bowel disease (IBD), haematological disorders or arthritis. The latest multicentric retrospective analysis published by Ghazal et al. shows that anaemia has been observed very often in German patients suffering from PG (in 45.6% of 259) so this disorder is supposed to be a possible cofactor in the pathogenesis of PG. According to its progressive course, patients require intensive diagnostic procedures and rapid initiation of the treatment. In this article, we report a case of bullous pyoderma gangrenosum in association with pancytopenia of unknown origin, according to its diagnostic and therapeutic difficulties.

摘要

坏疽性脓皮病(PG)是一种病因不明的嗜中性皮病。临床上,它始于脓疱、结节或水疱,迅速发展并演变成边界隆起、呈潜行性的疼痛性溃疡。PG的发病机制尚不清楚。然而,它常与系统性疾病相关,如炎症性肠病(IBD)、血液系统疾病或关节炎。Ghazal等人发表的最新多中心回顾性分析表明,在德国PG患者中经常观察到贫血(259例中有45.6%),因此这种疾病被认为可能是PG发病机制中的一个辅助因素。根据其进展过程,患者需要进行深入的诊断程序并迅速开始治疗。在本文中,根据其诊断和治疗困难,我们报告了一例与不明原因全血细胞减少相关的大疱性坏疽性脓皮病病例。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b0aa/4171674/f55bc6d1d097/PDIA-31-22386-g001.jpg

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