Abstract: WU Zheng-sheng*, JIN Liang, MA Dong-lai, YAN Heng, GENG Song-mei, CHEN Liu-qing, TAN De-you, LI Yong, YUAN Wei, YAN Ting-kai, YANG Lu-ting, ZHANG Wei, WANG Gang. *Department of Dermatology, Xijing Hospital, Fourth Military Medical University, Xi'an 710032, China Corresponding author: WANG Gang, Email: xjwgang@fmmu.edu.cn 【Abstract】 Objective To investigate the clinical and pathological features of pseudoxanthoma elasticum. Methods Clinical data on 29 cases of pseudoxanthoma elasticum were retrospectively collected. Clinical manifestations, treatment outcome and findings from histopathological examination, Verhoeff-van Gieson staining and von Kossa′s staining were studied. Results Among these patients, the ratio of male to female was 1 ∶ 8.7, with an average age at onset of （22. 6 ± 11.2） years. Lesions were distributed in the neck （29 cases, 100%）, axillary fossa （11 cases, 37.9%）, abdomen （5 cases, 17.2%）, or groin （4 cases, 13.8%）. Hematoxylin-eosin （HE） staining of lesions consistently revealed characteristic curl-like structures in mid dermis, which were confirmed to be swelling, disrupted, curled, and degenerated elastic fibers by Verhoeff-van Gieson staining. von Kossa′s staining showed calcium deposition on the degenerated elastic fibers. Of five patients receiving fundus oculi examination and cardiovascular system examination, two had angioid streaks radiating from the video disc, one even had mild retinal hemorrhage and vision impairment, none showed cardiovascular abnormalities. Hyperthyroidism was detected in one of the 29 patients. Conclusions Pseudoxanthoma elasticum has distinct clinical manifestations, and it is usually not difficult to diagnose pseudoxanthoma elasticum according to clinical manifestations as well as findings from histopathological examination and Verhoeff-van Gieson staining. 【Key words】 Pseudoxanthoma elasticum; Pathology; Diagnosis