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一名患有急性结节病并伴有发热、多关节炎和结节性红斑的患者:典型的 Löfgren 综合征

[A patient with acute sarcoidosis associated with fever, polyarthritis, and erythema nodosum: a typical of Löfgren's syndrome].

作者信息

Koseki Y, Terai C, Nakajima H, Tokuda H, Ichikawa N, Tsutsumino M, Nakajima A, Akama H, Taniguchi A, Hara M, Kashiwazaki S

机构信息

Institute of Rheumatology, Tokyo Women's Medical College.

出版信息

Ryumachi. 1998 Feb;38(1):23-8.

PMID:9564774
Abstract

A 26-year-old female was admitted to Aoyama Hospital in February 1996, for evaluation of abnormal chest shadows and polyarthritis. She visited our clinic in December 1995 with complaints of fever, fatigue, and polyarthralgia which lasted for 3 weeks. Two weeks later she developed erythema nodosum on her lower legs with the exacerbation of polyarthritis. Erythrocyte sedimentation rate was 108 mm/hr and CRP 4.9 mg/dl. A chest radiograph showed hilar lymphadenopathy and multiple nodular shadows in both lung fields. On admission, she had arthritis of the shoulders and knees, erythema nodosum on her lower extremities, and keloid-like skin lesion on her left knee. Thoracoscopic lung biopsy of nodular lesion and skin biopsy of keloid-like lesion revealed noncaseating granuloma of epithelioid cells with lymphocytes, macrophages, and giant cells, confirming the diagnosis of sarcoidosis. She was cured in three months, and was well and free of the symptoms thereafter. Löfgren's syndrome is acute sarcoidosis, characterized by arthritis, erythema nodosum, and bilateral hilar lymphadenopathy. This syndrome is common in Europe and is closely related to HLA-B8 and DR-3. The frequency of HLA-B8 and DR-3 in Japanese is almost 0%, explaining the rare onset of this syndrome in Japan. Our case is the second report of typical Löfgren's syndrome in Japan, although the patient did not have these HLA loci.

摘要

一名26岁女性于1996年2月入住青山医院,以评估异常胸部阴影和多关节炎。她于1995年12月前来我院门诊,主诉发热、乏力和多关节痛,持续了3周。两周后,随着多关节炎加重,她下肢出现结节性红斑。红细胞沉降率为108mm/小时,CRP为4.9mg/dl。胸部X线片显示肺门淋巴结肿大及双肺野多发结节状阴影。入院时,她有双肩和双膝关节炎、下肢结节性红斑以及左膝瘢痕疙瘩样皮肤病变。结节性病变的胸腔镜肺活检及瘢痕疙瘩样病变的皮肤活检显示上皮样细胞非干酪性肉芽肿,伴有淋巴细胞、巨噬细胞和巨细胞,确诊为结节病。她在三个月内治愈,此后情况良好且无任何症状。勒夫格伦综合征是急性结节病,其特征为关节炎、结节性红斑和双侧肺门淋巴结肿大。该综合征在欧洲很常见,且与HLA - B8和DR - 3密切相关。日本人中HLA - B8和DR - 3的频率几乎为0%,这解释了该综合征在日本发病罕见的原因。我们的病例是日本第二例典型勒夫格伦综合征报告,尽管该患者没有这些HLA位点。

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