摘要
Erdheim-Chester病是一种罕见的非朗格汉斯组织细胞疾病,可累及多系统,如骨关节、神经、皮肤、大血管及内分泌等。临床最常见表现为累及下肢关节周围的轻微骨痛。于2020年4月9日四川大学华西医院收治1例以多浆膜腔积液为首发表现、缺乏骨痛症状的患者,正电子发射计算机断层显像CT检查提示骨骼及大血管受累,最终经骨活检病理确诊为Erdheim-Chester病。该患者呈现出罕见病的不典型表现,通过对该病例的临床资料分析有助于提高临床医师对该病的识别。
Erdheim-Chester disease is a rare non-Langerhans histiocytic disease that can involve multiple systems,such as bone and joint,nerve,skin,large blood vessels and endocrine.The most common clinical manifestation is slight bone pain around the lower limb joints.The data reported a patient with multiple serous cavity effusion as the first manifestation and lack of symptoms of bone pain.PET-CT examination showed that the bone and large blood vessels were involved,and finally confirmed as Erdheim-Chester disease by bone biopsy pathology.The patient presented atypical manifestations of rare diseases,and the analysis of clinical data of the case will help improve the recognition of the disease by clinicians.
作者
缪治永
杨闵
岑筱敏
Miao Zhiyong;Yang Min;Cen Xiaomin(Laboratory of Human Disease and Immunotharepy,West China Hospital,Sichuan University,Chengdu 610041,China;Department of Rheumatology,West China Hospital,Sichuan University,Chengdu 610041,China)
出处
《中国综合临床》
2023年第2期122-125,共4页
Clinical Medicine of China
