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一例可能存在家族性发病的德库姆病的鉴别诊断,并文献复习。

Differential Diagnosis of a Case of Dercum's Disease with Possible Familial Involvement and Review of Literature.

机构信息

University of Queensland/Ochsner Clinical School, Ochsner Clinic Foundation, New Orleans, LA, USA.

Department of Medicine, Stamford Hospital, Columbia College of Physicians and Surgeons, Stamford, CT, USA.

出版信息

Yale J Biol Med. 2021 Dec 29;94(4):603-608. eCollection 2021 Dec.

Abstract

Dercum's disease (DD), also described as adiposis dolorosa, is a poorly understood and rare adipose tissue disorder involving obesity and painful adipose tissue masses. Patients may have associated bruising and constitutional symptoms such as fatigue, difficulty concentrating, and sleep disturbance. DD was initially described in 1888 by Francis Xavier Dercum, and was classified into four subtypes, including generalized diffuse, generalized nodular, localized nodular, and juxta-articular subtypes. While this disease has been described for more than 130 years, its etiology and treatment remain elusive. We describe a case of a patient with DD who presented to Ochsner Medical Center, New Orleans, LA, for evaluation of treatment options. We review current knowledge on this rare disease and data on modern treatment methods.

摘要

德库姆病(DD),也称为痛性肥胖病,是一种了解甚少且罕见的脂肪组织疾病,涉及肥胖和疼痛性脂肪组织肿块。患者可能伴有瘀斑和全身症状,如疲劳、注意力难以集中和睡眠障碍。DD 最初由 Francis Xavier Dercum 于 1888 年描述,并分为四个亚型,包括全身性弥漫型、全身性结节型、局限性结节型和关节旁型。虽然这种疾病已经被描述了 130 多年,但它的病因和治疗仍然难以捉摸。我们描述了一例 DD 患者在新奥尔良 Ochsner 医疗中心就诊,以评估治疗方案。我们回顾了这种罕见疾病的现有知识和现代治疗方法的数据。

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[Dercum's disease].
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[A case of adiposis dolorosa--Dercum's disease].
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本文引用的文献

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Differentiating lipedema and Dercum's disease.鉴别脂肪性水肿和德卡姆病。
Int J Obes (Lond). 2017 Feb;41(2):240-245. doi: 10.1038/ijo.2016.205. Epub 2016 Nov 18.
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Syndromic lipomatosis of the head and neck: a review of the literature.头颈部综合征性脂肪瘤病:文献综述
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