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一个诊断难题:急性皮肤型红斑狼疮模仿中毒性表皮坏死松解症的不寻常表现。

A Diagnostic Conundrum: Unusual Presentation of Acute Cutaneous Lupus Erythematosus Mimicking Toxic Epidermal Necrolysis.

作者信息

Kamble Nilesh, Khot Rajashree, Barai Saransh, Chaudhari Sachin, Rathod Bharatsing, Awadhiya Onkar

机构信息

Department of General Medicine, All India Institute of Medical Sciences, Nagpur, India.

出版信息

Eur J Rheumatol. 2025 Mar 24;12(1):1-4. doi: 10.5152/eurjrheum.2025.24030.

Abstract

Systemic lupus erythematosus (SLE) is an intricate autoimmune disorder with diverse clinical presentations, encompassing various cutaneous manifestations. This case report describes a diagnostically challenging occurrence of acute cutaneous lupus erythematosus (ACLE) exhibiting a toxic epidermal necrolysis (TEN)-like rash in a 28-year-old female already diagnosed with SLE. The patient's rapid progression from itching to maculopapular skin eruptions involving the face, extremities, and torso within days, coupled with facial puffiness and systemic symptoms, presented a clinical conundrum. Histopathological findings of epidermal hyperkeratosis, acanthosis, and a subcorneal neutrophilic abscess guided the exclusion of other conditions, emphasizing the distinctive features of TEN-like ACLE. The patient exhibited a favorable response to pulse methylprednisolone, mycophenolate mofetil, and hydroxychloroquine.

摘要

系统性红斑狼疮(SLE)是一种复杂的自身免疫性疾病,临床表现多样,包括各种皮肤表现。本病例报告描述了一名28岁已确诊为SLE的女性患者出现的具有诊断挑战性的急性皮肤型红斑狼疮(ACLE),其表现为中毒性表皮坏死松解症(TEN)样皮疹。患者在数天内迅速从瘙痒发展为累及面部、四肢和躯干的斑丘疹皮肤疹,同时伴有面部肿胀和全身症状,这构成了一个临床难题。表皮角化过度、棘层肥厚和角质层下中性粒细胞脓肿的组织病理学发现有助于排除其他疾病,突出了TEN样ACLE的独特特征。患者对脉冲甲基强的松龙、霉酚酸酯和羟氯喹表现出良好反应。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0b82/12012747/ad8f153d5b85/ejr-12-1-24030_f001.jpg

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