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广泛特发性坏疽性脓皮病从溃疡型演变为增殖型:10年病史及近期对英夫利昔单抗的反应

Widespread idiopathic pyoderma gangrenosum evolved from ulcerative to vegetative type: a 10-year history with a recent response to infliximab.

作者信息

Marzano A V, Tourlaki A, Alessi E, Caputo R

机构信息

Institute of Dermatological Sciences, University of Milan, Fondazione IRCCS Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena, Milan, Italy.

出版信息

Clin Exp Dermatol. 2008 Mar;33(2):156-9. doi: 10.1111/j.1365-2230.2007.02607.x. Epub 2007 Nov 15.

Abstract

Pyoderma gangrenosum (PG) is an infrequent neutrophilic dermatosis, which commonly presents with a limited number of ulcerative, pustular, bullous or vegetative lesions associated with an underlying systemic disorder. We report a 34-year-old man with ulcerative PG that was exceptionally widespread and not associated with any other condition. Moreover, it was resistant to steroid treatment and, after prolonged use of ciclosporin, it unexpectedly developed a vegetative pattern, further supporting the hypothesis that the different forms of PG are part of a single clinical spectrum. Finally, dramatic improvement of the condition occurred after treatment with infliximab, an antitumour necrosis factor-alpha monoclonal antibody; however, this produced circulating autoantibodies. Although this has not had any clinical consequence to date, accurate follow-up in patients treated with infliximab is essential to monitor the onset of a possible autoimmune disorder induced by the drug.

摘要

坏疽性脓皮病(PG)是一种罕见的嗜中性皮肤病,通常表现为数量有限的溃疡性、脓疱性、大疱性或增殖性皮损,且与潜在的全身性疾病相关。我们报告一名34岁患有溃疡性PG的男性,其皮损异常广泛且与任何其他疾病无关。此外,该患者对类固醇治疗耐药,在长期使用环孢素后,意外出现增殖性皮损,这进一步支持了不同形式的PG是单一临床谱系一部分的假说。最后,使用抗肿瘤坏死因子-α单克隆抗体英夫利昔单抗治疗后病情显著改善;然而,这产生了循环自身抗体。尽管迄今为止这尚未产生任何临床后果,但对接受英夫利昔单抗治疗的患者进行准确随访对于监测该药物可能诱发的自身免疫性疾病的发生至关重要。

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