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伴有弹性假黄瘤样微观改变的瘤样脂性水肿。

Tumefactive lipedema with pseudoxanthoma elasticum-like microscopic changes.

作者信息

Taylor Nancy E, Foster William C, Wick Mark R, Patterson James W

机构信息

Department of Pathology, University of Virginia Health System, Charlottesville, VA, USA.

出版信息

J Cutan Pathol. 2004 Feb;31(2):205-9. doi: 10.1111/j.0303-6987.2004.00168.x.

DOI:10.1111/j.0303-6987.2004.00168.x
PMID:14690469
Abstract

BACKGROUND

Lipedema is a condition characterized by diffuse, bilaterally symmetrical, painful swelling of the legs and buttocks. Microscopically, there are dermal and septal edema, adipocyte degeneration, and numerous mast cells, features held in common with lipedematous alopecia.

CASE REPORT

We present the case of a 60-year-old woman with a long history of bilateral leg masses with microscopic features of lipedema. In addition, elastic-fiber changes typical of pseudoxanthoma elasticum (PXE) were discovered within the subcutaneous septa in three separate specimens obtained from an affected extremity. The patient did not have other clinical findings of PXE, although there was a history of both hypertension and congestive heart failure.

CONCLUSION

This tumefactive presentation of lipedema has not been previously described. Regarding the elastic-tissue abnormalities, the patient could have either a subclinical form of PXE, perhaps predisposing to lipedema, or secondary elastic-tissue changes resulting from the massive edema. If the latter is the case, then this could represent an unusual manifestation of localized acquired cutaneous PXE (calcific elastosis).

摘要

背景

脂肪性水肿是一种以双下肢及臀部弥漫性、双侧对称性、疼痛性肿胀为特征的病症。显微镜下可见真皮及间隔水肿、脂肪细胞变性以及大量肥大细胞,这些特征与脂肪性水肿性脱发相同。

病例报告

我们报告一例60岁女性患者,其双侧腿部肿物病史较长,具有脂肪性水肿的微观特征。此外,在取自患侧肢体的三个独立标本的皮下间隔内发现了弹性假黄瘤(PXE)典型的弹性纤维改变。尽管患者有高血压和充血性心力衰竭病史,但并无PXE的其他临床症状。

结论

这种脂肪性水肿的肿胀表现此前未见报道。关于弹性组织异常,该患者可能患有PXE的亚临床形式,或许这是导致脂肪性水肿的原因,或者是由大量水肿引起的继发性弹性组织改变。如果是后者,那么这可能代表局限性后天性皮肤PXE(钙化弹性组织变性)的一种不寻常表现。

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Tumefactive lipedema with pseudoxanthoma elasticum-like microscopic changes.伴有弹性假黄瘤样微观改变的瘤样脂性水肿。
J Cutan Pathol. 2004 Feb;31(2):205-9. doi: 10.1111/j.0303-6987.2004.00168.x.
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[Pseudoxanthoma elasticum-like papillary dermal elastolysis: A case report].[弹性假黄瘤样乳头真皮弹力纤维松解症:1例报告]
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Calciphylaxis with pseudoxanthoma elasticum-like changes: A case series.伴有弹性假黄瘤样改变的钙过敏症:病例系列
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Pseudoxanthoma elasticum-like change adjacent to a benign adnexal neoplasm: a histopathologic reaction pattern.良性附属器肿瘤旁的弹性假黄瘤样改变:一种组织病理学反应模式
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A yellow plaque with keratotic papules on the abdomen. Perforating calcific elastosis (periumbilical perforating pseudoxanthoma elasticum [PXE], localized acquired cutaneous PXE).腹部有一个伴有角化丘疹的黄色斑块。穿通性钙化弹性组织变性(脐周穿通性弹性假黄瘤[PXE],局限性获得性皮肤PXE)。
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Dermoscopic features of pseudoxanthoma elasticum.弹性假黄瘤的皮肤镜表现。
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Cerebral small vessel disease in pseudoxanthoma elasticum: three cases.弹性假黄瘤中的脑小血管疾病:三例报告
Can J Neurol Sci. 2005 Feb;32(1):115-8. doi: 10.1017/s0317167100016991.

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