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伴弥漫性肺疾病的无肌病性皮肌炎

Amyopathic dermatomyositis with diffuse lung disease.

作者信息

Bandi Sumeera, Jain Vidushi, Jaiswal Ashish K

机构信息

Department of Pulmonary Medicine, HIMSR, Delhi, India.

Department of Dermatology, Dermalinks, Ghaziabad, Uttar Pradesh, India.

出版信息

J Family Med Prim Care. 2020 Jun 30;9(6):3147-3150. doi: 10.4103/jfmpc.jfmpc_106_20. eCollection 2020 Jun.

Abstract

A 38-year-old woman presented with progressively increasing breathlessness, recurrent productive cough, and intermittent fever of 1 year duration. Examination revealed cutaneous eruptions on the dorsal aspects of the hands and on face. Histopathologic features of skin biopsy revealed acanthosis, hyperkeratosis with focal vacuolar alteration of the basal-cell layer, and perivascular inflammatory infiltrates in upper dermis. CT scan showed diffuse lung disease and pulmonary function tests showed severe restrictive lung disease. There was no muscular involvement clinically or on electromyography and magnetic resonance imaging. She was diagnosed as a case of amyopathic dermatomyositis with diffuse lung disease and managed with topical and systemic steroid and topical sunscreen with fairly good response.

摘要

一名38岁女性,出现进行性加重的呼吸困难、反复咳痰性咳嗽及持续1年的间歇性发热。检查发现双手背及面部有皮肤疹。皮肤活检的组织病理学特征显示棘层肥厚、角化过度伴基底细胞层局灶性空泡改变,以及真皮上层血管周围炎性浸润。CT扫描显示弥漫性肺部疾病,肺功能测试显示严重的限制性肺病。临床、肌电图及磁共振成像均未发现肌肉受累。她被诊断为合并弥漫性肺部疾病的无肌病性皮肌炎,并接受局部和全身类固醇治疗以及局部使用防晒霜,反应良好。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dcd1/7491809/9e0782bab52e/JFMPC-9-3147-g001.jpg

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