一个罕见的侵袭性坏疽脓皮病与科根综合征在一个人的皮肤颜色。

Q3 Medicine
Skin health and disease Pub Date : 2025-02-14 eCollection Date: 2025-02-01 DOI:10.1093/skinhd/vzae027
Nageswary Nadarajah, Lucy Clark, Shernaz Walton
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引用次数: 0

摘要

Cogan综合征(CS)是一种罕见的自身免疫性血管炎,影响听觉前庭和眼部系统。其发病机制尚不清楚。1980年将CS分为典型CS和非典型CS,以辅助诊断。其与坏疽性脓皮病(PG)的关系在文献中仅报道过三次。这也是第一例出现在有色人种身上的病例。CS是一种排除性诊断,因此其诊断可能会给医疗保健专业人员带来许多挑战。在这里,我们描述的情况下,一个75岁的南亚妇女谁提出了急性中风后,右脑腔隙性梗塞,用阿司匹林和氯吡格雷治疗。在最近的全左髋关节置换术中发现了一个扩大的未愈合的伤口。开始静脉注射抗生素,并进行多次手术清创。入院时,胸部和腹部出现两个新的疼痛脓疱性皮损,并在2天内溃烂。左侧臀部伤口和臀部两个受压区也可见疼痛的溃疡性病变,边缘呈蓝色。临床诊断为PG,并开始使用大剂量皮质类固醇治疗,但没有改善。患者既往病史包括左眼视网膜中央静脉阻塞伴复发性葡萄膜炎和双侧感音神经性耳聋。诊断为不典型CS。四次脉冲环磷酰胺输注和高压氧治疗病变。该病例表明PG和CS之间复杂的相互作用,需要进一步研究,因为它可能导致显著的发病率。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
A rare case of aggressive pyoderma gangrenosum with Cogan syndrome in a person with skin of colour.

Cogan syndrome (CS) is a rare autoimmune vasculitis affecting the audiovestibular and ocular systems. Its pathogenesis is unknown. CS was classified into typical and atypical CS in 1980 to aid its diagnosis. Its association with pyoderma gangrenosum (PG) has only been reported three times in the literature. This is also the first case of its occurrence in a person with skin of colour. CS is a diagnosis of exclusion and thus its diagnosis may present many challenges to healthcare professionals. Herein, we describe the case of a 75-year-old South Asian woman who presented acutely to the Stroke Unit following a right lacunar infarction which was treated with aspirin and clopidogrel. An enlarging nonhealing wound was noted at the site of a recent total left hip replacement. Intravenous antibiotics were started, with multiple surgical debridements performed. During admission, two new painful pustular skin lesions erupted on the chest and abdomen that ulcerated within 2 days. Painful ulcerated lesions with bluish undermined edges were also noted at the left hip wound and two pressure areas of the buttocks. A clinical diagnosis of PG was made and treatment was started with high-dose corticosteroids, which did not lead to improvement. The patient's past medical history included left eye central retinal vein occlusion with recurrent uveitis and bilateral sensorineural deafness. A diagnosis of atypical CS was made. Four pulsed cyclophosphamide infusions and hyperbaric oxygen healed the lesions. This case demonstrates the complex interplay between PG and CS, which requires further research as it can result in significant morbidity.

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来源期刊
CiteScore
1.70
自引率
0.00%
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审稿时长
10 weeks
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