Benign Symmetric Lipomatosis in Korean Patients

Background: Madelung’s disease (MD) is characterized by multiple symmetric deposits of unencapsulated adipose tissue in the head, neck, and upper trunk. However, the etiology of lipoma tissue in MD remains controversial. Objective: This study determined the clinicopathological and epidemiological features of Korean patients with MD and re-examined the hypothesis that lipomas of MD originate from brown fat. Methods: We performed a single-institution, retrospective medical record review of 20 patients diagnosed with MD between January 1997 and August 2017. Biopsy slides were stained with nuclear factor IA (NFIA) and uncoupling protein 1 (UCP-1). Results: The patients included were 2 women and 18 men with an average age of 64 (range, 27∼75) years. Ten patients reported heavy alcohol intake, and the remaining 10 reported some degree of alcohol intake. Hepatic disease was present in 47.7% of patients, only 16.7% had a body mass index ＞30 kg/㎡, and 80% underwent surgical intervention. Conclusion: MD affected mainly alcoholic men in the fourth decade. Alcoholism, hepatic disease, or severe obesity accounted for less than half of the Korean patients with MD. Immunohistochemical staining supported the hypothesis that MD lipomas originate from brown fat. Although the adipose tissue of all patients was stained with NFIA and UCP-1, the staining intensity varied. NFIA, which is a transcription factor required for the induction and maintenance of brown fat-specific gene expression, was more sensitive than UCP-1 for the detection of brown fat. (Korean J Dermatol 2020;58(2):92∼96)