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一名苏丹患者抗MDA - 5抗体阳性无肌病性皮肌炎的皮肤特征

Cutaneous Features of Anti-MDA-5 Antibody-Positive Amyopathic Dermatomyositis in a Sudanese Patient.

作者信息

Alqahtani Nouf, Aleissa Majed

机构信息

Division of Dermatology, King Abdulaziz medical city, Ministry of National Guard Health Affairs, Riyadh, Saudi Arabia.

College of Medicine, King Saud bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia.

出版信息

Case Rep Dermatol. 2021 Sep 28;13(3):481-485. doi: 10.1159/000519104. eCollection 2021 Sep-Dec.

DOI:10.1159/000519104
PMID:34720922
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8543314/
Abstract

Clinically amyopathic dermatomyositis (CADM) is a rare form of DM characterized by unique cutaneous and pulmonary features with no muscle involvement. A subset of patients with CADM has a specific antibody known as anti-melanoma differentiation-associated protein 5 (MDA5). The systemic associations of anti-MDA-5 CADM warrant an early recognition and management to prevent fetal sequelae. It is seen more commonly in white and Asian female individuals. The clinical features of anti-MDA5 antibody-positive CADM in other ethnic groups are not well reported. Here, we describe a case of CADM with identified autoantibodies against MDA5 in a Sudanese female patient presenting with characteristic cutaneous features in association with MDA5 autoantibodies: ulcerated Gottron's papules, painful palmar papules, shawl sign, and heliotrope sign. No evidence of pulmonary or systemic involvement was identified. Treatment with prednisolone and mycophenolate mofetil was initiated. This case emphasizes the importance of keeping a high level of suspicion and to recognize the unique clinical feature of this type of DM aiding in early treatment and preventing fatal outcomes.

摘要

临床上,无肌病性皮肌炎(CADM)是皮肌炎的一种罕见形式,其特征为具有独特的皮肤和肺部表现,而无肌肉受累。一部分CADM患者有一种名为抗黑色素瘤分化相关蛋白5(MDA5)的特异性抗体。抗MDA-5 CADM的全身关联情况需要早期识别和处理,以预防不良后果。它在白人和亚洲女性个体中更为常见。关于其他种族中抗MDA5抗体阳性CADM的临床特征报道较少。在此,我们描述一例苏丹女性CADM患者,该患者检测出抗MDA5自身抗体,伴有与MDA5自身抗体相关的特征性皮肤表现:Gottron丘疹溃疡、掌部疼痛性丘疹、披肩征和向阳疹。未发现肺部或全身受累的证据。开始使用泼尼松龙和霉酚酸酯进行治疗。该病例强调了保持高度怀疑并认识到这类皮肌炎独特临床特征对于早期治疗和预防致命后果的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d57c/8543314/af64d2e1456c/cde-0013-0481-g02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d57c/8543314/e60d5e3a6196/cde-0013-0481-g01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d57c/8543314/af64d2e1456c/cde-0013-0481-g02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d57c/8543314/e60d5e3a6196/cde-0013-0481-g01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d57c/8543314/af64d2e1456c/cde-0013-0481-g02.jpg

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本文引用的文献

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JAAD Case Rep. 2020 Jul 22;6(9):909-911. doi: 10.1016/j.jdcr.2020.07.019. eCollection 2020 Sep.
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Dermatomyositis: Clinical features and pathogenesis.皮肌炎:临床特征与发病机制。
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Mycophenolate mofetil for the patients with interstitial lung diseases in amyopathic dermatomyositis with anti-MDA-5 antibodies.霉酚酸酯用于抗MDA - 5抗体阳性无肌病性皮肌炎合并间质性肺疾病的患者。
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RNA helicase encoded by melanoma differentiation-associated gene 5 is a major autoantigen in patients with clinically amyopathic dermatomyositis: Association with rapidly progressive interstitial lung disease.黑色素瘤分化相关基因5编码的RNA解旋酶是临床无肌病性皮肌炎患者的主要自身抗原:与快速进展性间质性肺病的关联。
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