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非典型结节性皮肤狼疮黏蛋白病一例报告

A case report of atypical nodular cutaneous lupus mucinosis.

作者信息

Wang Na, Shan Xiaofeng, Wu Weizhi, Shen Xiaoting, Hu Tim Xiaoming, Pei Zhenhuan, Wang Keyu

机构信息

Qilu Hospital of Shandong University, Jinan Shandong Provincial Institute of Dermatology and Venereology, Jinan, Shandong First Affiliated Hospital of Sun Yat-sen University, Guangzhou, Guangdong, P. R. China Harvard Medical School, Harvard University, Boston, Massachusetts, USA.

出版信息

Medicine (Baltimore). 2017 Dec;96(49):e8391. doi: 10.1097/MD.0000000000008391.

Abstract

RATIONAL

Nodular cutaneous lupus mucinosis is regarded as a distinctive cutaneous mucinosis deposition with systemic lupus erythematosus(SLE). All typical cases occurred as asymptomatic cutaneous papules, nodules, or plaques on the trunk, upper and lower extremities, and face. Histopathology is mainly revealed abundant mucin deposits among splayed collagen bundles in the dermis. At the same time we can find A the typical clinical manifestations and biological evidence of SLE. Here, we report the first case of nodular cutaneous lupus mucinosis that did not present with any prior symptoms or history of SLE.

PATIENT CONCERNS

We report the first case of nodular cutaneous lupus mucinosis that did not present with any prior symptoms or history of SLE. The patient was 34 years old. One year before admission, nodules began to appear on the elbows, chest, and back, and 2 months before admission erythema occurred on the face. Other notable clinical symptoms were not observed and had no prior history of SLE.

DIAGNOSES

Initially, this patient was misdiagnosed by other clinics as having eczema. After histopathological assessment of skin biopsy and examination of antinuclear antibody signals, the patient was correctly diagnosed with nodular cutaneous lupus mucinosis.

INTERVENTIONS

Followed administration of systemic steroids and hydroxychloroquine.

OUTCOMES

the eruptions quickly disappeared and laboratory indicators improved.

LESSONS

This case highlights the need for diagnostic vigilance in cases involving papules and nodules initially developing on the chest and elbows in the absence of obvious lupoid symptoms. We recommend a lower threshold for performing histopathological analysis and examination of antinuclear antibody signals in view of the rare but serious possibility of nodular cutaneous lupus mucinosis.

摘要

理论依据

结节性皮肤狼疮黏蛋白病被认为是一种伴有系统性红斑狼疮(SLE)的独特的皮肤黏蛋白沉积症。所有典型病例均表现为躯干、上下肢及面部出现无症状的皮肤丘疹、结节或斑块。组织病理学主要显示真皮层中展开的胶原束间有大量黏蛋白沉积。同时我们能发现SLE的典型临床表现和生物学证据。在此,我们报告首例无任何SLE既往症状或病史的结节性皮肤狼疮黏蛋白病病例。

患者情况

我们报告首例无任何SLE既往症状或病史的结节性皮肤狼疮黏蛋白病病例。患者34岁。入院前一年,肘部、胸部和背部开始出现结节,入院前2个月面部出现红斑。未观察到其他明显临床症状,且无SLE既往病史。

诊断

最初,该患者被其他诊所误诊为湿疹。经皮肤活检的组织病理学评估及抗核抗体信号检查后,患者被正确诊断为结节性皮肤狼疮黏蛋白病。

干预措施

随后给予全身用类固醇和羟氯喹治疗。

结果

皮疹迅速消退,实验室指标改善。

经验教训

该病例强调了对于最初在胸部和肘部出现丘疹和结节且无明显狼疮样症状的病例进行诊断时需保持警惕。鉴于结节性皮肤狼疮黏蛋白病虽罕见但严重的可能性,我们建议降低进行组织病理学分析和抗核抗体信号检查的阈值。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8fb6/5728828/e9ebeddf229c/medi-96-e8391-g001.jpg

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