结节病伴普遍严重关节局部化1例。

IF 2.3
Multidisciplinary Respiratory Medicine Pub Date : 2016-06-29 eCollection Date: 2016-01-01 DOI:10.1186/s40248-016-0064-1
Livio G Moccia, Sabrina Castaldo, Emanuela Sirignano, Maddalena Napolitano, Enrica Barra, Alessandro Sanduzzi
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引用次数: 2

摘要

背景:结节病是一种来源不明的全身性肉芽肿性疾病,其特征是肉芽肿的形成没有中心坏死。每个器官和组织都可受到疾病的影响,但在大多数情况下主要是肺和纵隔淋巴结,但也包括皮肤、心脏、眼睛和关节,后者主要是掌指关节和骨病变通常与上覆皮肤受累有关。诊断通常是排除性的,基于临床和放射学的怀疑,并应通过活检证实,尽管在每个病例中都有必要排除其他可能的肉芽肿病原因,包括分枝杆菌感染。本文报告一例侵袭性结节病,原发累及手脚小关节和纵隔淋巴结。病例介绍:患者为男性,60岁,摩洛哥出生,意大利居住多年,主要累及骨结构和关节周围软组织,并伴有肉芽肿形成,无“干酪样坏死”。疼痛症状和皮肤溃疡导致手术截肢其手和脚的大多数手指的远端指骨,但随后手术后在肢端部位复发病变。PET/CT扫描显示纵隔淋巴结可见一定量的示踪剂,而TBNA取样淋巴结正常,支气管肺泡灌洗液CD4/CD8比值小于3。我们排除了任何可能的感染原因,包括分枝杆菌感染(结核性和非典型),因此患者接受全身皮质类固醇治疗,临床和放射学反应良好。结论:该病例显示了该疾病如何具有可变表达,而没有原始肺部受累;因此,有必要考虑任何可能的、不可预测的疾病定位。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Sarcoidosis with prevalent and severe joint localization: a case report.

Sarcoidosis with prevalent and severe joint localization: a case report.

Sarcoidosis with prevalent and severe joint localization: a case report.

Sarcoidosis with prevalent and severe joint localization: a case report.

Background: Sarcoidosis is a systemic granulomatous disease of unknown origin, characterized by the formation of granulomas without central necrosis. Each organ and tissue can be affected by the disease, but in most cases mainly the lungs and mediastinal lymph nodes but also skin, heart, eyes and joints are involved, the latter are mainly the metacarpophalangeal joints and bone lesions are often associated with involvement of the overlying skin. The diagnosis is often of exclusion, based on clinical and radiological suspicion, and should be confirmed by biopsy, although in each case it is necessary to exclude other possible causes of granulomatosis, including infections by mycobacteria. Here it is reported a case of particularly aggressive sarcoidosis with primitive involvement of the small joints of the hands and feet, and mediastinal lymph nodes.

Case presentation: The subject, a man, 60 years old, born in Morocco but living in Italy for many years, presented important involvement of bone structures and soft periarticular tissue, and was affected by the formation of granulomas without "caseum necrosis". The painful symptoms and the skin ulceration had led to surgical amputation of the distal phalanges of most fingers of his hands and feet, but with subsequent resurgence of lesions in acral locations after surgery. The PET/CT scan showed an amount of radiotracer in mediastinal lymph nodes, while the lymph nodes sampled by TBNA were normal and the CD4/CD8 ratio was less than 3 in the bronchoalveolar lavage. We ruled out any possible infectious cause, including mycobacterial infection (both tubercular and atypical), so the patient was treated with systemic corticosteroids, with an excellent clinical and radiological response.

Conclusions: Such a case shows how the disease can have variable expressions, without primitive lung involvement; therefore, it should be necessary to consider any possible, unpredictable localization of the disease.

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来源期刊
Multidisciplinary Respiratory Medicine
Multidisciplinary Respiratory Medicine Medicine-Pulmonary and Respiratory Medicine
自引率
0.00%
发文量
23
期刊介绍: Multidisciplinary Respiratory Medicine is the official journal of the Italian Respiratory Society - Società Italiana di Pneumologia (IRS/SIP). The journal publishes on all aspects of respiratory medicine and related fields, with a particular focus on interdisciplinary and translational research. The interdisciplinary nature of the journal provides a unique opportunity for researchers, clinicians and healthcare professionals across specialties to collaborate and exchange information. The journal provides a high visibility platform for the publication and dissemination of top quality original scientific articles, reviews and important position papers documenting clinical and experimental advances.
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