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家族性皮肤胶原瘤

Familial cutaneous collagenoma.

作者信息

Dawn Marianne E, Deng April C, Petrali John, Wessely Caroline, Jaffe David, Gaspari Anthony A

机构信息

Department of Dermatology, University of Maryland School of Medicine, Baltimore, MD 21201, USA.

出版信息

Skinmed. 2008 Jan-Feb;7(1):43-5. doi: 10.1111/j.1540-9740.2007.06353.x.

DOI:10.1111/j.1540-9740.2007.06353.x
PMID:18174804
Abstract

A 41-year-old woman presented to our dermatology clinic in February 2005 with a chief complaint of numerous flesh-colored nodules on her back and abdomen. She initially noticed the lesions at age 17 years. The plaques had increased in size and number over time, but remained asymptomatic. The patient reported multiple similar lesions on a maternal uncle and a cousin. Her family history was also notable for cardiomyopathy, resulting in the death of her mother. The patient's past medical history was notable for poorly controlled type I diabetes, currently managed with an insulin pump; and coronary artery disease. The patient had undergone multiple cardiac procedures before the age of 40 years, including quadruple coronary artery bypass grafting surgery and placement of 9 cardiac stents. Her ejection fraction on cardiac catheterization in November 2004 was 65% with no wall motion abnormalities. On physical examination, numerous spongy, discrete, flesh-colored plaques and nodules were seen concentrated across the upper part of her back between the scapulae as well as underneath the breasts and across the flanks (Figure 1). All lesions were asymptomatic. Prior workup of this patient had included plain films of the long bones and hands, which were within normal limits. A biopsy from lesional skin on the back highlighted by trichome stain showed an increased number of markedly thickened and eosinophilic dermal collagen bundles compared with adjacent normal skin. Immunohistochemical studies with anticollagen type I and type III antibodies confirmed that the increased collagen material consisted of type I collagen fibers, which is the same type of collagen found in normal dermis. The elastic fibers, highlighted by Verhoeff-van Gieson stain (Figure 2), were diminished and haphazardly arranged. No increased cellular component or inflammatory infiltrate was observed. These findings were consistent with a collagenoma. Further analysis of the lesional tissue by electron microscopy revealed that the ultrastructural appearance of the collagen fibers, including arrangement and diameters, were not significantly different from that of the normal tissue (Figure 3).

摘要

2005年2月,一名41岁女性因背部和腹部出现大量肤色结节为主诉前来我院皮肤科就诊。她最初在17岁时发现这些皮损。随着时间推移,斑块的大小和数量均有增加,但仍无症状。患者报告其一位舅舅和一位表亲有多处类似皮损。她的家族史还因心肌病导致其母亲死亡而引人注目。患者既往病史以I型糖尿病控制不佳(目前使用胰岛素泵治疗)及冠状动脉疾病为显著特点。该患者在40岁之前接受过多次心脏手术,包括冠状动脉搭桥术和9个心脏支架植入术。2004年11月心脏导管检查时她的射血分数为65%,无室壁运动异常。体格检查发现,大量海绵状、散在的肤色斑块和结节集中分布于双侧肩胛间背部上方、乳房下方及双侧胁腹部(图1)。所有皮损均无症状。该患者之前的检查包括长骨和手部X线平片,结果均正常。背部皮损皮肤活检经苏木精-伊红染色显示,与相邻正常皮肤相比,真皮胶原束明显增厚且嗜酸性增强,数量增多。用抗I型和III型胶原抗体进行免疫组织化学研究证实,增加的胶原物质由I型胶原纤维组成,与正常真皮中发现的胶原类型相同。用Verhoeff-van Gieson染色显示的弹性纤维(图2)减少且排列紊乱。未观察到细胞成分增加或炎症浸润。这些发现符合胶原瘤。对皮损组织进行电子显微镜进一步分析显示,胶原纤维的超微结构外观,包括排列和直径,与正常组织无明显差异(图3)。

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