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皮肤狼疮黏蛋白病的非典型表现。

Atypical presentation of cutaneous lupus mucinosis.

作者信息

Goerig Ryan, Vogeler Chad, Keller Matthew

机构信息

Thomas Jefferson University Hospital, Department of Dermatology and Cutaneous Biology, Philadelphia, Pennsylvania.

出版信息

J Clin Aesthet Dermatol. 2013 Apr;6(4):37-40.

Abstract

OBJECTIVE

To describe the epidemiological, clinical, and serological features of cutaneous lupus mucinosis and its relationship to systemic lupus erythematosus as well as elucidate the histopathological features of cutaneous lupus mucinosis and describe how these features differ from tumid lupus erythematosus.

DESIGN

Case review and assessment of the literature.

SETTING

University academic setting.

PARTICIPANT

One patient.

RESULTS

The authors report a case of antinuclear antibody negative cutaneous lupus mucinosis in a patient without systemic involvement who responded to hydroxychloroquine and intralesional triamcinolone. A review of the literature found 30 reported cases of cutaneous lupus mucinosis, three of which were antinuclear antibody negative and the majority had systemic lupus erythematosus. The most common therapy reported is systemic corticosteroids; however, the patient described in this case had significant improvement of the lesions with only intralesional steroid injections and hydroxychloroquine. Considering the proportion of patients with cutaneous lupus mucinosis who progress to systemic lupus is uncertain, the authors suggest following these patients closely for evidence of multisystem disease.

CONCLUSION

The authors report a case of antinuclear antibody-negative cutaneous lupus mucinosis in a patient without systemic lupus erythematosus who responded to hydroxychloroquine and intralesional triamcinolone. Given the rarity of this condition and reported association with systemic lupus erythematosus, it is important to follow these patients clinically for any signs or symptoms of systemic involvement.

摘要

目的

描述皮肤狼疮黏蛋白病的流行病学、临床和血清学特征及其与系统性红斑狼疮的关系,并阐明皮肤狼疮黏蛋白病的组织病理学特征,以及描述这些特征与肿胀性红斑狼疮有何不同。

设计

病例回顾及文献评估。

地点

大学学术环境。

参与者

一名患者。

结果

作者报告了一例抗核抗体阴性的皮肤狼疮黏蛋白病患者,该患者无全身受累,对羟氯喹和皮损内注射曲安奈德有反应。文献回顾发现30例报告的皮肤狼疮黏蛋白病病例,其中3例抗核抗体阴性,大多数患者患有系统性红斑狼疮。报告的最常见治疗方法是全身使用糖皮质激素;然而,本病例中描述的患者仅通过皮损内注射类固醇和羟氯喹,皮损就有显著改善。考虑到皮肤狼疮黏蛋白病患者进展为系统性狼疮的比例尚不确定,作者建议密切随访这些患者,以寻找多系统疾病的证据。

结论

作者报告了一例无系统性红斑狼疮的抗核抗体阴性皮肤狼疮黏蛋白病患者,该患者对羟氯喹和皮损内注射曲安奈德有反应。鉴于这种情况罕见且与系统性红斑狼疮有关联,临床上密切随访这些患者以发现任何全身受累的体征或症状很重要。

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引用本文的文献

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本文引用的文献

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A case of nodular cutaneous lupus mucinosis.
J Dermatol. 2003 Apr;30(4):341-3. doi: 10.1111/j.1346-8138.2003.tb00398.x.
7
Cutaneous mucinoses: microscopic criteria for diagnosis.皮肤黏蛋白沉积症:诊断的微观标准
Am J Dermatopathol. 2001 Jun;23(3):257-67. doi: 10.1097/00000372-200106000-00022.
8
Cutaneous lupus mucinosis: a review of our cases and the possible pathogenesis.
J Cutan Pathol. 1997 Oct;24(9):553-8. doi: 10.1111/j.1600-0560.1997.tb01459.x.

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