德克姆氏病:复发性脂肪瘤需要手术治疗的独特病例

Taylor W. Young , Geoffrey Zhang , Linwood R. Haith Jr.
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摘要

Dercum病,又称多脂性脂肪症,是一种罕见的疾病,其特征是多发性脂肪瘤引起可触及的皮肤缺陷,伴有疼痛和压痛。这种疾病的流行和病因仍然未知,尽管它对受影响的个体有衰弱的影响。目前的文献描述患有德克姆氏病的个体主要是老年肥胖女性。本报告详细介绍了一个独特的病例,涉及一名29岁的男性,在2022年至2024年期间,他在五个不同的场合接受了80多个脂肪瘤的手术切除。手术前,在门诊对结节进行评估,以确定其位置,确认皮肤变化,并评估其对患者生活质量的削弱作用,从而证明手术方法是合理的。组织学证实所有病变均为良性脂肪瘤。在最初的脂肪瘤切除后,躯干和四肢又复发了新的病变。根据家族史、病变部位和组织学排除神经纤维瘤病、多发性家族性脂肪瘤病、Madelung病、Bannayan-Zonana综合征和Cowden综合征等鉴别诊断。尽管自19世纪后期以来,德克姆氏病已经被发现,但在了解和治疗这种疾病方面的进展有限。这个病例的目的是阐明一个年轻的病人的经验,多发性复发脂肪瘤需要手术。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Dercum's disease: A unique case of recrudescent lipomas necessitating surgery
Dercum’s disease, alternatively known as adiposis dolorosa, is a rare condition characterized by multiple lipomas causing palpable skin defects associated with pain and tenderness. The prevalence and etiology of this disease remain unknown despite its debilitating impact on affected individuals. Current literature describes individuals with Dercum’s disease to be predominantly older, obese females. This report details a unique case involving a 29-year-old male who, between 2022 and 2024, underwent surgical excision of over 80 lipomas on five separate occasions. Prior to surgery, the nodules were assessed in an outpatient setting to determine their location, confirm skin changes, and evaluate their debilitating effects on the patient’s quality of life, thereby justifying the surgical approach. Histology proved all the lesions to be benign lipomas. Subsequent to the initial lipoma excisions, new lesions recurred on the torso and extremities. Differential diagnoses were considered such as neurofibromatosis, multiple familial lipomatosis, Madelung disease, Bannayan-Zonana syndrome, and Cowden syndrome were ruled out by family history, location of lesions, and histology. Although Dercum’s disease has been recognized since the late 1800s, advances in understanding and treating the condition have been limited. This case aims to shed light on the experience of a young patient with multiple recrudescent lipomas necessitating surgery.
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