Oueriagli Nabih Salah
Tunis Med. 2016 Jun;94(6):167-171.
The Erdheim-Chester disease is a rare form of non Langherans histiocytosis. It can be expressed by a multi systemic disease interesting: bone, lung, heart, brain, retro peritoneum, skin and retro-orbital tissue. The diagnosis is established under the radiological aspect, bone scan uptake and immunohistological profile. The bone lesions are almost constant and rather characteristic of bone scintigraphy. We related the case of a young female patient of 26 years old with Erdheim-Chester bone disease. Diagnosis was revealed by diabetes insipidus (nerve damage) and diffuses bones pain. Histological confrontation was obtained on a biopsy, guided by abnormalities described in bone scan. The evolution under interferon was marked by clinical and scintigraphic worsening. Through this clinical observation we will discuss the interest of bone scintigraphy exploration in the monitoring of this disease under a therapy most often not codified.
厄尔德海姆-切斯特病是一种罕见的非朗格汉斯组织细胞增多症。它可表现为一种累及多系统的疾病,涉及骨骼、肺、心脏、脑、腹膜后、皮肤和眶后组织。诊断基于放射学表现、骨扫描摄取情况和免疫组织学特征。骨病变几乎是恒定的,且在骨闪烁显像中颇具特征性。我们报告了一例26岁患有厄尔德海姆-切斯特骨病的年轻女性患者。诊断由尿崩症(神经损伤)和弥漫性骨痛揭示。在骨扫描所描述的异常引导下,通过活检获得了组织学对照。在干扰素治疗下,病情进展表现为临床和闪烁显像结果恶化。通过这一临床观察,我们将讨论在这种通常没有规范治疗方案的疾病治疗过程中,骨闪烁显像检查的作用。