皮肤红斑狼疮的罕见中毒性表皮坏死症样表现:6例系列病例。

IF 1.9 4区 医学 Q3 RHEUMATOLOGY
Lupus Pub Date : 2024-08-01 Epub Date: 2024-05-14 DOI:10.1177/09612033241255266
Varun Rajagopal, Anupama Bains, Karthick Kannan, Abhishek Bhardwaj, Poonam Elhence
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引用次数: 0

摘要

TEN 类红斑狼疮是红斑狼疮中一种不常见的危及生命的变异型。它通常与系统性红斑狼疮的复发有关,而且由于广泛的皮肤糜烂,还可能导致急性皮肤衰竭。它常与药物诱发的 TEN 相混淆,但两种疾病的治疗方法不同,因此正确诊断至关重要。在这项研究中,我们旨在评估印度人群中类似 LE 的 TEN 的临床特征和预后。我们对所有符合 ACR/EULAR 2019 系统性红斑狼疮标准、临床诊断为 TEN-like LE 的患者进行了回顾性研究。共确定了 6 名患者。所有患者均为女性。除1名患者为新发病例外,其他患者在发病前均存在结缔组织病症状。半数患者有掌跖受累。粘膜受累程度较轻。大多数患者的全身受累表现为肾炎,其次是关节痛、自身免疫性肝炎和自身免疫性溶血性贫血。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Uncommon toxic epidermal necrolysis -like presentation of cutaneous lupus erythematosus: A series of 6 cases.

TEN like Lupus erythematosus is an uncommon life-threatening variant of Lupus erythematosus. It is usually associated with flares of systemic lupus erythematosus and also because of widespread skin erosions, it can cause acute skin failure. It is often confused with drug induced TEN, however the management of both the diseases is different and hence correct diagnosis becomes crucial. In this study we aimed to assess the clinical characteristics and outcome of TEN like LE in the Indian population. All patients satisfying ACR/EULAR 2019 criteria for SLE and clinically diagnosed with TEN like LE were retrospectively reviewed. A total of 6 patients were identified. All patients were female. Except 1 patient who presented de-novo, the others had pre-existing symptoms of connective tissue disease. Half of the patients had palmoplantar involvement. Mucosal involvement was only mild. Majority had systemic involvement in the form of nephritis followed by arthralgia, autoimmune hepatitis and autoimmune hemolytic anemia.

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来源期刊
Lupus
Lupus 医学-风湿病学
CiteScore
4.20
自引率
11.50%
发文量
225
审稿时长
1 months
期刊介绍: The only fully peer reviewed international journal devoted exclusively to lupus (and related disease) research. Lupus includes the most promising new clinical and laboratory-based studies from leading specialists in all lupus-related disciplines. Invaluable reading, with extended coverage, lupus-related disciplines include: Rheumatology, Dermatology, Immunology, Obstetrics, Psychiatry and Cardiovascular Research…
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