文献简介

出版社:JAMA Dermatology

作  者:Eray Yihui Zhou, MD; Yi Zhao, MD; Zhimiao Lin, MD

编  号:

关键字:Vascular Ehlers-Danlos syndrome ;elastosis perforans serpiginosa

年  份:2017   点击量:336

文献摘要 全文翻译

A young woman presented with multiple arciform to annular plaques on her extremities, favoring the antecubital and popliteal fossae (Figure, A). The patient reported that these asymptomatic lesions had been present since her adolescence, and she was easily bruised with minimal trauma since birth. There was no family history of similar cutaneous findings.

患者年轻女性,四肢处出现多个弧状至环状斑块,有向肘前和肘窝处发展趋势(图A)。患者自诉自青春期起这些无症状皮损一直存在,且自出生以来患者极易受伤。无类似皮肤病家族史。

 

On close inspection, the plaques were found to be composed of individual 2- to 4-mm keratotic papules surrounding white atrophic patches. Physical examination also revealed remarkable skin atrophy on the dorsal aspects of her hands (Figure, B) and feet.The patient’s skin was generally pale and translucent with visible veins, especially on the chest and abdomen. The face appeared to be emaciated, with a pinched nose, thin lips, sunken cheeks, and prominent eyes. A punch biopsy specimen from an individual papule from the left arm was obtained and analyzed under Verhoeff–van Gieson stain (Figure, C).

进一步检查发现,斑块由单个2 至4 mm角化性丘疹构成,周围绕以白色萎缩斑。体格检查发现患者手背和脚部皮肤显著萎缩(图B)。患者皮肤通常苍白,半透明,可见静脉,尤其是胸部和腹部皮肤。患者面部憔悴、鼻子皱缩、嘴唇薄、面颊凹陷、眼睛凸出。从左手臂单个丘疹处取钻孔活检标本,并进行Verhoeff van Gieson染色分析(图C)。