一种罕见的误导性疾病：埃尔德海姆-切斯特病的孤立性骨骼受累

IF 11.4 1区医学 Q1 RHEUMATOLOGY

Arthritis & Rheumatology Pub Date : 2024-10-21 DOI:10.1002/art.43039

Sara Bindoli, Stefania Vio, Marta Sbaraglia, Fabrizio Vianello, Roberta Ramonda

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引用次数: 0

摘要

埃尔德海姆-切斯特病（Erdheim-Chester Disease，ECD）是一种非朗格汉斯组织细胞增生症，由泡沫组织细胞在多个器官和系统中的异常聚集引起。我们的患者已经患有骨质疏松症，双侧踝关节剧烈疼痛并伴有功能障碍，临床表现类似于 "复杂性区域疼痛综合征"（CRPS）。由于孤立的骨定位在 ECD 中并不常见，因此诊断具有挑战性。

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A rare and misleading condition: isolated skeletal involvement of Erdheim-Chester Disease

We are describing an unusual presentation of Erdheim-Chester Disease (ECD), a non -Langerhans histiocytosis provoked by abnormal aggregation of foamy histiocytes in several organs and systems. Our patient, already with osteoporosis, presented severe bilateral ankle pain with functional impairment, clinically mimicking “Complex Regional Pain Syndrome” (CRPS); the radiological images and subsequently the bone biopsy were fundamental for achieving the proper diagnosis. The diagnosis was challenging given the isolated bone localization which is uncommonly seen in ECD.

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来源期刊

Arthritis & Rheumatology RHEUMATOLOGY-

CiteScore

20.90

自引率

3.00%

发文量

371

期刊介绍： Arthritis & Rheumatology is the official journal of the American College of Rheumatology and focuses on the natural history, pathophysiology, treatment, and outcome of rheumatic diseases. It is a peer-reviewed publication that aims to provide the highest quality basic and clinical research in this field. The journal covers a wide range of investigative areas and also includes review articles, editorials, and educational material for researchers and clinicians. Being recognized as a leading research journal in rheumatology, Arthritis & Rheumatology serves the global community of rheumatology investigators and clinicians.