巨脂性营养障碍：局部巨大症的罕见病因。

Macrodystrophia Lipomatosa: An Unusual Cause of Localized Gigantism.

作者信息

Maheswari S Uma, Sampath V, Ramesh A, Manoharan K

机构信息

Department of Dermatology, Madras Medical College and Rajiv Gandhi Government General Hospital, Chennai, Tamil Nadu, India.

出版信息

Indian J Dermatol. 2016 May-Jun;61(3):347. doi: 10.4103/0019-5154.182469.

DOI:10.4103/0019-5154.182469

PMID:27293271

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4885204/

Abstract

Macrodystrophia lipomatosa (MDL) is a rare congenital form of localized gigantism characterized by progressive overgrowth of all mesenchymal elements with a disproportionate increase in fibro adipose tissue. Here we report a case of 20 years old male who presented with history of painless gradual enlargement of entire left upper limb since childhood. Magnetic resonance imaging and histopathology confirmed the diagnosis of macrodystrophia lipomatosa. This condition has to be differentiated from other causes of localized gigantism, since these conditions differ in their course, prognosis, complications and treatment.

摘要

巨脂性营养障碍（MDL）是一种罕见的先天性局限性巨人症，其特征是所有间充质成分进行性过度生长，纤维脂肪组织不成比例增加。我们在此报告一例20岁男性患者，自童年起即有左侧上肢无痛性逐渐增大的病史。磁共振成像和组织病理学确诊为巨脂性营养障碍。这种情况必须与其他局限性巨人症的病因相鉴别，因为这些情况在病程、预后、并发症及治疗方面有所不同。