Madelung's Disease: Analysis of Clinical Characteristics, Fatty Mass Distribution, Comorbidities and Treatment of 54 Patients in China

Runze Li; Chuanxiao Wang; Quandong Bu; Wenshen Pu; Bin Zhou; Lin Che; Hui Zhang; Yan Xu; Hong Luan

doi:10.2147/DMSO.S363836

Madelung's Disease: Analysis of Clinical Characteristics, Fatty Mass Distribution, Comorbidities and Treatment of 54 Patients in China

Diabetes Metab Syndr Obes. 2022 Aug 6:15:2365-2375. doi: 10.2147/DMSO.S363836. eCollection 2022.

Authors

Runze Li^#^{1

2}, Chuanxiao Wang^#³, Quandong Bu⁴, Wenshen Pu⁵, Bin Zhou⁴, Lin Che⁴, Hui Zhang⁴, Yan Xu⁴, Hong Luan⁴

Affiliations

¹ Department of Medicine, Qingdao University, Qingdao, People's Republic of China.
² Department of Endocrine, Affiliated Hospital of Qingdao University, Qingdao, People's Republic of China.
³ Department of Thoracic Surgery, Qingdao Municipal Hospital, Qingdao, People's Republic of China.
⁴ Department of Nephrology, Affiliated Hospital of Qingdao University, Qingdao, People's Republic of China.
⁵ Department of Nephrology, Baoshan People's Hospital, Baoshan, People's Republic of China.

^# Contributed equally.

Abstract

Purpose: Madelung's disease (MD) is a rare disease characterized by the deposition of unencapsulated fat masses on the face, neck, chest, back and other areas of patients. The aim of the study was to analyze the clinical characteristics, comorbidities and treatment of MD in Chinese populations.

Patients and methods: We retrospectively reviewed the medical records of 54 patients who were diagnosed with MD at the Affiliated Hospital of Qingdao University and Qingdao Municipal Hospital from January 2005 to February 2021 and collected the subjects' demographic information, clinical indicators, location of fat deposits, treatment, complications and prognostic data.

Results: Among 54 MD patients in the study, only 1 (1.85%) was female, and the subjects had an average age of 56.65 ± 7.93 years. More than 70% of patients had a history of long-term smoking or/and alcohol abuse. In our study, type I accounted for approximately 61.11% of cases according to Donhauser's classification, and almost all patients had neck fat deposition. MD patients often have multiple comorbidities across several systems, such as the endocrine, digestive, circulatory, urinary, and neurological systems. Among these, endocrine system diseases were the most common comorbidities in our study, accounting for 81.48%. Notably, up to 20.37% of cases were complicated with cancer, especially digestive system tumors. More than 70% of the patients received surgical treatment, and nearly 40% experienced postoperative recurrence.

Conclusion: Considering that MD patients often have comorbidities of multiple systems and that a small number of cases are even complicated by cancer, we recommend that clinicians comprehensively assess a patient's condition and complications, advocate that patients quit consuming alcohol and smoking as soon as possible, establish healthy dietary and living habits, and formulate individualized and comprehensive diagnosis and treatment plans.

Keywords: Launois-Bensaude syndrome; Madelung’s disease; alcohol abuse; benign symmetric lipomatosis; multiple symmetric lipomatosis.

Grants and funding

This work was supported by grants from the National Natural Science Foundation of Shandong Province (ZR2020QH060), the National Natural Science Foundation of China (81770679, 81470973, and 81970582), and the Qingdao Key Health Discipline Development Fund. No funding bodies had any role in the study design, data collection, and analysis, decision to publish, or preparation of the manuscript.