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混合性结缔组织病综合征

Mixed connective tissue disease syndrome.

作者信息

Gilliam J N, Prystowsky S D

出版信息

Arch Dermatol. 1977 May;113(5):583-7.

PMID:67824
Abstract

Fifteen patients with epidermal nuclear staining on direct immunofluorescence of normal skin and high titer serum antibody to ribonuclease-sensitive extractable nuclear antigen (ENA) had diffuse nonscarring and focal alopecia, abnormal pigmentation, swollen hands with sclerodactyly, and chronic cutaneous lupus erythematosus (LE) as the most common dermatologic features. Direct immunofluorescence of normal, unexposed skin revealed a particulate ('speckled') epidermal nuclear staining pattern in all 15 patients and subepidermal immunoglobulin deposits in 5. Ribonucleoprotein antibodies in high titer are associated with this characteristic type of epidermal nuclear staining. These findings provide easily detectable markers for a less aggressive subset of LE characterized by distinctive clinical and laboratory features consistent with mixed connective tissue disease.

摘要

15例正常皮肤直接免疫荧光显示表皮核染色且抗核糖核酸酶敏感的可提取核抗原(ENA)血清抗体滴度高的患者,最常见的皮肤表现为弥漫性非瘢痕性和局灶性脱发、色素沉着异常、手部肿胀伴指硬皮病以及慢性皮肤型红斑狼疮(LE)。对正常未暴露皮肤进行直接免疫荧光检查发现,所有15例患者均有颗粒状(“斑点状”)表皮核染色模式,5例有表皮下免疫球蛋白沉积。高滴度核糖核蛋白抗体与这种特征性的表皮核染色类型相关。这些发现为一种侵袭性较小的LE亚组提供了易于检测的标志物,其具有与混合性结缔组织病一致的独特临床和实验室特征。

相似文献

1
Mixed connective tissue disease syndrome.混合性结缔组织病综合征
Arch Dermatol. 1977 May;113(5):583-7.
2
[Nucleolar, homogeneous and peripheral fluorescence of epidermal nuclei in direct immunofluorescence: 4 cases (author's transl)].直接免疫荧光法检测表皮细胞核仁、均匀及周边荧光:4例(作者译)
Ann Dermatol Venereol. 1979 Apr;106(4):361-4.
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Direct immunofluorescent findings in scleroderma syndromes.硬皮病综合征的直接免疫荧光检查结果。
Acta Derm Venereol. 1981;61(1):29-36.
5
[Sharp's syndrome: a new entity amongst the connective tissue diseases?].[夏普综合征:结缔组织病中的一种新病症?]
Nouv Presse Med. 1975 Jun 21;4(25):1863-5.
6
Association of antibodies to ribonucleoprotein and Sm antigens with mixed connective-tissue disease, systematic lupus erythematosus and other rheumatic diseases.核糖核蛋白和Sm抗原抗体与混合性结缔组织病、系统性红斑狼疮及其他风湿性疾病的关联。
N Engl J Med. 1976 Nov 18;295(21):1149-54. doi: 10.1056/NEJM197611182952101.
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Mixed connective tissue disease.混合性结缔组织病
Arch Dermatol. 1976 Nov;112(11):1535-8.
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Epidermal nuclear immunoglobulin deposition in connective tissue diseases.结缔组织病中的表皮核免疫球蛋白沉积
Rev Hosp Clin Fac Med Sao Paulo. 1990 Jul-Aug;45(4):154-7.
9
[The Sharp syndrome ("mixed connective tissue disease")].[夏普综合征(“混合性结缔组织病”)]
Schweiz Med Wochenschr. 1976 Jan 31;106(5):129-34.
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[The clinical significance of soluble nuclear antigen specific antibodies (author's transl)].可溶性核抗原特异性抗体的临床意义(作者译)
Nouv Presse Med. 1978 May 27;7(21):1813-8.

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6
Mixed connective tissue disease.混合性结缔组织病
West J Med. 1980 Apr;132(4):288-93.
7
Budd-Chiari syndrome in a patient with mixed connective-tissue disease.一名混合性结缔组织病患者的布加综合征。
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Clinical profiles of patients with antibodies to nuclear ribonucleoprotein.抗核糖核蛋白抗体患者的临床特征
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Systemic sclerosis. The Watson Smith Lecture 1984.系统性硬化症。1984年沃森·史密斯讲座
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Morphoea (localised scleroderma) in a patient with mixed connective tissue disease.一名混合性结缔组织病患者出现硬斑病(局限性硬皮病)。
Ann Rheum Dis. 1986 Jun;45(6):523-5. doi: 10.1136/ard.45.6.523.