ターナー症候群(45 X/46 XX/47 XXXのモザイク)に発症した全身性エリテマトーデスの1例  [in Japanese] A Case of Systemic Lupus Erythematosus Assosiated with Turner's Syndrome  [in Japanese]

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Author(s)

    • 辻 卓夫 TSUJI Takuo
    • 名古屋市立大学医学部皮膚科学教室 Department of Dermatology, Nagoya City University Medical School

Abstract

38歳の女性。15年前から筋力低下,関節痛,レイノー症状があり,多発性筋炎として治療を受けていた。その後,手指関節の変形,体幹の網状暗赤色斑,熱発が出現し,精査のため入院。白血球2900/<I>μ</I>l,抗核抗体陽性,lupus band test陽性,抗DNA抗体(RIA)7730IU/<I>μ</I>lと著明高値を示し,眼科,口唇生検,唾液腺造影の所見とあわせ,シェーグレン症候群を合併した全身性エリテマトーデスと診断した。低身長(141cm)があり,末梢血リンパ球の染色体分析で45X/46XX/47XXXのモザイクを示すことから,ターナー症候群と考えられた。両者の合併は,自己免疫疾患の発症要因を検討する上で注目に値すると考えた。

A 38-year-old woman with Turner's syndrome associated with systemic lupus erythematosus (SLE) and Sjögren syndrome is reported. She had been diagnosed to have polymyositis, because she has been suffering from arthralgia, Raynaud's phenomenon and muscle weakness for 15 years. In 1992 she was referred to our Dermatology clinic due to the development of high fever, reticular erythema, and a modification of the joint. The laboratory findings revealed leukopenia, a positive LE cell test and high titers of antinuclear and anti-DNA antibodies. Direct immunofluorescence revealed IgG and IgM deposition in the basement membrane. She was short (141cm) and her karyotype was found to be 45X/46XX/47XXX according to a lymphocyte chromosome study. Based on these findings, she was diagnosed to have SLE with Turner's syndrome.

Journal

  • The Nishinihon Jourrnal of Dermatology  

    The Nishinihon Jourrnal of Dermatology 64(2), 157-160, 2002-04-01 

    Western Division of Japanese Dermatological Association

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