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一例以指端溃疡和坏疽为首发表现的儿童系统性红斑狼疮罕见病例。

A Rare Case of Digital Ulceration and Gangrene as an Initial Presentation of Systemic Lupus Erythematosus in a Child.

作者信息

Vengala Shyam, Gupta Varnika, Kandukuri Vaishnavi, Nuthalapati Bala Sai Teja, Pillikunte Doddareddy Navya, Raj Deepanshu, Parmar Mihirkumar P, Venugopal Vishal

机构信息

Internal Medicine, Government Medical College, Srikakulam, Visakhapatnam, IND.

Internal Medicine, Lala Lajpat Rai Memorial Medical College, Meerut, IND.

出版信息

Cureus. 2023 Nov 12;15(11):e48698. doi: 10.7759/cureus.48698. eCollection 2023 Nov.

Abstract

Systemic lupus erythematosus (SLE) is a complex autoimmune illness with a wide range of symptoms. Tissue-binding autoantibodies and intricate immune complexes are responsible for the initial damage to organs and cellular structures. Dermatological signs, particularly digital gangrene and ulcers, are uncommon in the context of systemic lupus erythematosus and often appear in the advanced stages of the disease. In this discussion, we present an unusual example of early-onset digital gangrene and ulcers in a young kid with systemic lupus erythematosus. It is unusual because SLE is mostly seen in adult patients, but here the patient is a seven-year-old boy who went to the doctor because he had urticarial rashes all over his body and face, skin desquamation, and sporadic fever episodes. The preliminary evaluation had difficulty separating this presentation from acute urticaria. However, further diagnostic testing and serological analysis confirmed the patient's SLE diagnosis. The distal regions of the fingers developed digital gangrene, ulceration, and vasculitis. Clinical and serological tests were used to confirm the diagnosis. Antinuclear antibodies (ANA), anti-ribonuclear protein (Anti-RNP) antibodies, anti-Smith (Anti-Sm) antibodies, and anti-Sjögren's syndrome-related antigen A (Anti-SS-A) antibodies were all positive in the patient. This example emphasizes the critical need to recognize the unusual and severe signs of SLE in medical practice.

摘要

系统性红斑狼疮(SLE)是一种具有广泛症状的复杂自身免疫性疾病。组织结合自身抗体和复杂的免疫复合物是导致器官和细胞结构初始损伤的原因。皮肤症状,特别是指端坏疽和溃疡,在系统性红斑狼疮患者中并不常见,且常出现在疾病的晚期。在本讨论中,我们呈现了一例患有系统性红斑狼疮的幼儿出现早发性指端坏疽和溃疡的不寻常病例。此病例不寻常之处在于系统性红斑狼疮多见于成年患者,而这里的患者是一名七岁男孩,他因全身及面部出现荨麻疹样皮疹、皮肤脱屑和散发性发热而去看医生。初步评估难以将此表现与急性荨麻疹区分开来。然而,进一步的诊断测试和血清学分析证实了该患者的系统性红斑狼疮诊断。手指远端出现了指端坏疽、溃疡和血管炎。通过临床和血清学检查确诊。该患者的抗核抗体(ANA)、抗核糖核蛋白(Anti-RNP)抗体、抗史密斯(Anti-Sm)抗体和抗干燥综合征相关抗原A(Anti-SS-A)抗体均呈阳性。这个病例强调了在医疗实践中识别系统性红斑狼疮不寻常和严重症状的迫切需要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7adf/10715716/eff9e096ac9e/cureus-0015-00000048698-i01.jpg

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