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乳头真皮弹力纤维增生症:一种独特的弹性组织疾病还是弹性假黄瘤样乳头真皮弹力纤维溶解症的不寻常表现?

Papillary dermal elastosis: a unique elastic tissue disorder or an unusual manifestation of pseudoxanthoma elasticum-like papillary dermal elastolysis?

作者信息

Wang Annie R, Lewis Kevan, Lewis Michelle, Robinson-Bostom Leslie

机构信息

Department of Dermatology, Rhode Island Hospital, Warren Alpert Medical School of Brown University, Providence, RI 02903, USA.

出版信息

J Cutan Pathol. 2009 Sep;36(9):1010-3. doi: 10.1111/j.1600-0560.2008.01183.x. Epub 2008 Dec 18.

Abstract

There are numerous acquired elastic tissue disorders, several of which present cutaneously with small yellow-to-white papules resembling plucked chicken skin. Differential diagnoses depend on the abnormalities within the network of elastic tissues. We report a case with distinct histologic features, which may represent a unique elastic tissue disorder or a variant of pseudoxanthoma elasticum-like papillary dermal elastolysis. Our patient's clinical presentation includes scattered 1-2 mm white-to-yellow papules without surface change on the upper back and neck region. Histology is characterized by foci of clumped, granular elastic tissue, which have replaced the oxytalan and elaunin fibers, alternating with foci of decreased concentrations of normal-appearing elastic fibers within the papillary dermis. Given its characteristics, we have termed this novel entity 'papillary dermal elastosis'.

摘要

有许多获得性弹性组织疾病,其中几种在皮肤上表现为类似拔毛鸡皮的小黄白色丘疹。鉴别诊断取决于弹性组织网络内的异常情况。我们报告了一例具有独特组织学特征的病例,这可能代表一种独特的弹性组织疾病或弹性假黄瘤样乳头真皮弹力纤维溶解症的一种变体。我们患者的临床表现为上背部和颈部区域散在分布的1 - 2毫米白色至黄色丘疹,表面无变化。组织学特征为成簇的颗粒状弹性组织灶,其取代了氧化弹性纤维和弹力素纤维,与乳头真皮内正常外观弹性纤维浓度降低的区域交替出现。鉴于其特征,我们将这种新实体命名为“乳头真皮弹力纤维病”。

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