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结节病作为对称性巨大疱疹样皮肤病的罕见病因。

Sarcoidosis as a rare cause for symmetrical giant bullous disease.

机构信息

Department of Thoracic Surgery, University Hospital Zurich, Raemistrasse 100, 8091, Zurich, Switzerland.

Department of Thoracic Surgery, Medical University Brandenburg, Campus Neuruppin, Germany.

出版信息

BMC Pulm Med. 2017 May 30;17(1):88. doi: 10.1186/s12890-017-0429-z.

Abstract

BACKGROUND

Sarcoidosis presents with typical clinic-radiological findings and shows histologically non-caseating granulomas. Pulmonary manifestations of sarcoidosis can be diverse, involving the intrathoracic lymph nodes and pulmonary parenchyma.

CASE PRESENTATION

We here describe a case of a 35-year-old patient who presented with a history of exertion dyspnoea and coughing for the past 20 years. At the age of 15, she was exposed to smoke emanating from a fire. Later, she had exposure to mold for two years, and during her childhood, she had animals such as a cockatiel, dog, cat, gecko, and turtle. Computed tomography of the chest revealed symmetrical apical giant bullous lesions. Histology of the resected bullae showed prominent peribronchial fibrosis with non-necrotizing, non-caseating granulomas and collaps of pulmonary lobules adjacent to the bulla. The absence of granulomatous infection and a markedly elevated CD4:CD8 ratio in bronchoalveolar lavage analysis suggested that the underlying process was sarcoidosis.

CONCLUSION

In very rare cases, sarcoidosis can be associated with bilateral symmetrical apical giant bullous disease due to fibrotic and granulomatous changes resulting in a restriction of lung tissue.

摘要

背景

结节病表现为典型的临床影像学表现,并显示出组织学上无干酪样坏死的肉芽肿。结节病的肺部表现可以是多种多样的,累及胸内淋巴结和肺实质。

病例介绍

我们在此描述了一例 35 岁患者的病例,该患者有 20 年的劳累性呼吸困难和咳嗽史。15 岁时,她曾接触过火灾产生的烟雾。后来,她又接触了两年的霉菌,在童年时期,她还养过鸡尾鹦鹉、狗、猫、壁虎和乌龟。胸部计算机断层扫描显示对称的肺尖大疱性病变。切除大疱的组织学显示明显的支气管周围纤维化,伴有非坏死性、非干酪样坏死的肉芽肿,以及与大疱相邻的肺小叶塌陷。支气管肺泡灌洗分析中无肉芽肿性感染和 CD4:CD8 比值明显升高,提示潜在过程是结节病。

结论

在非常罕见的情况下,由于纤维化和肉芽肿性改变导致肺组织受限,结节病可与双侧对称肺尖大疱性疾病相关。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/186a/5450335/cc076d863351/12890_2017_429_Fig1_HTML.jpg

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