匐行性穿通性弹性纤维病并发唐氏综合征  

A case of elastosis perforans serpiginosa complicated with Down’s syndrome

作　　者：白璐[1] 孙爱娟 王美红 王涛[2] 鞠玲燕 朱才勇[1] BAI Lu;SUN Aijuan;WANG Meihong;WANG Tao;JU Lingyan;ZHU Caiyong(Department of Dermatology,the Hospital of NO.80 Group Army,Weifang261021,China;Department of Pathology,the Hospital of NO.80 Group Army,Weifang261021,China)

机构地区：[1]中国人民解放军陆军第八十集团军医院皮肤病科,山东潍坊261021 [2]中国人民解放军陆军第八十集团军医院病理科,山东潍坊261021

出　　处：《临床皮肤科杂志》2024年第6期370-372,共3页Journal of Clinical Dermatology

摘　　要：患者男,23岁。四肢环状红丘疹3年。自幼有唐氏综合征病史。皮肤科检查:双肘关节、右膝关节及左大腿伸侧可见排列成环形或弓形的暗红色和棕褐色质硬丘疹,部分丘疹表面有白色鳞屑、凹陷及角栓,环形丘疹中央可见褐色羊皮纸样萎缩性斑片。右膝部丘疹组织病理:表皮棘细胞增生,可见角栓形成,角栓内及表皮基底层均见嗜碱性蓝染组织团块,由变性的上皮细胞、炎细胞碎片和弹性纤维组成。Masson染色:胶原纤维染色呈亮绿色,基底层变性的碎片状弹性纤维呈红色。诊断:匐行性穿通性弹性纤维病;唐氏综合征。治疗:他扎罗汀凝胶外用,3个月后病情好转。A 23-year-old male presented with annular red papules on the limbs for more than three years.He had a history of Down’s syndrome since childhood.Dermatological examination showed dark red and brownish hard papules with a diameter of 2-5 mm arranged in a circular and arched shape on the extended side of both elbows,right knee joint,and left thigh.Some scales were white,while others had indentations and angle plugs.Brown parchment like atrophic patches could be seen in the center of the circular shape.Pathology analysis of papular tissue on the right knee showed proliferation of epidermal spinous cells,visible formation of angle thrombi,and alkaline blue stained tissue masses inside the angle thrombi and at the base of the epidermis,composed of degenerated epithelial cells,inflammatory cell fragments,and elastic fibers.Masson staining showed bright green collagen fibers and red fragmented elastic fibers with degeneration at the base.In conclusion,it was diagnosed as elastosis perforans serpiginosa and Down’s syndrome.After giving tazarotene gel for external use once a night for three months,the condition was relieved.

关 键 词：匐行性穿通性弹性纤维病 唐氏综合征 

分 类 号：R758.69[医药卫生—皮肤病学与性病学] R442.8[医药卫生—临床医学]