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关节过度活动综合征患者的局灶性线性弹性组织变性。

Focal linear elastosis in a patient with joint hypermobility syndrome.

作者信息

Clement Barak C, Forster Christopher, Logemann Nicholas

机构信息

Walter Reed National Military Medical Center, Bethesda, Maryland.

出版信息

Dermatol Online J. 2018 Jan 15;24(1):13030/qt6n9456xv.

Abstract

Focal linear elastosis (FLE) is a benign skin findingcharacterized by hypertrophic linear plaques withabnormal elastic fibers on histology. We present aunique case in which focal linear elastosis occurredin the setting of joint hypermobility syndrome(JHS). Our patient, a 20-year-old man with a medicalhistory significant for symptoms consistent with JHS,had been followed by the rheumatology clinic formany months. He was referred to the dermatologydepartment for further evaluation of asymptomaticlongitudinal bands on his back that had been presentfor many years. He denied trauma but endorsed ahistory of 'stretch marks.' On examination there werenumerous horizontally oriented, firm, linear, yellowto flesh colored bands, all non-tender to palpation.Punch biopsies were performed of involved anduninvolved skin. Histopathology of normal skinrevealed no significant abnormalities whereasinvolved skin demonstrated broadened collagenbundles in the deep dermis. The elastic fiber stain,Verhoeff-Van Gieson, revealed a gross increase in thenumber of elastic fibers, fragmented fibers, fiberswith "paintbrush" or widened-ends, fibers of varyingthickness, and clumped fibers. This combination ofhistopathologic and clinical features was consistent with FLE.

摘要

局灶性线性弹性组织变性(FLE)是一种良性皮肤表现,其特征为肥厚性线性斑块,组织学检查显示弹性纤维异常。我们报告了一例独特的病例,局灶性线性弹性组织变性发生在关节活动过度综合征(JHS)的背景下。我们的患者是一名20岁男性,有与JHS相符症状的重要病史,在风湿病诊所随访了数月。他因背部多年来一直存在的无症状纵向条纹被转诊至皮肤科进一步评估。他否认有外伤史,但认可有“妊娠纹”病史。检查发现有许多水平方向的、坚硬的、线性的、黄色至肉色的条纹,触诊均无压痛。对受累和未受累皮肤进行了打孔活检。正常皮肤的组织病理学检查未发现明显异常,而受累皮肤在真皮深层显示胶原束增宽。弹性纤维染色(Verhoeff-Van Gieson染色)显示弹性纤维数量明显增加、纤维断裂、有“画笔样”或末端增宽的纤维、粗细不一的纤维以及成束的纤维。这种组织病理学和临床特征的组合符合局灶性线性弹性组织变性。

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