Madelung's Disease

摘要

The condition is also known as benign or multiple symmetric lipomatosis, or the Launois-Bensaude syn- drome. Two types of Madelung’s disease have been de- scribed. In type 1, fat accumulates around the neck and the nape of the neck, shoulders, upper arms, and upper back. In type 2, lipomas are distributed over much of the body, including the hips and thighs. Fatty deposits rarely extend to the lower limbs, mediastinum, and larynx in either type (2). Although the pathophysiology remains elusive, Mad- elung’s disease may be caused by a local defect in cate- cholamine-induced lipolysis (3). Most patients have a his- tory of chronic alcoholism. Mediterranean men appear to be at highest risk of acquiring the condition, whereas the disease is remarkably rare in Asian populations (3). The diagnosis of Madelung’s disease is primarily based on physical examination, clinical history, and imaging stud- ies. Diabetes mellitus (DM), lipid disorders, liver disease, and hypothyroidism are frequent comorbidities (4). Neu- ropathy, including sensory, motor, and autonomic poly- neuropathy, is observed in about 85% of patients, and the latter is associated with sudden cardiac death (5).