Madelung's Disease: Comorbidities, Fatty Mass Distribution, and Response to Treatment of 22 Patients

Madelung's disease (MD) is an uncommon pathology characterized by the presence of multiple masses of unencapsulated adipose tissue that is symmetrically distributed. The aim of this study was to investigate clinical and epidemiological features of patients diagnosed with MD in our department. Associated diseases and evolution after treatment were also investigated. We reviewed the clinical histories of 22 patients diagnosed with MD from 1990 to 2010 and obtained their epidemiological and clinical characteristics. We found 21 patients with MD type 1 and one patient with MD type 2 according to Enzi's classification. All patients were male, 95.5% with high alcohol intake, and 59.1% with some hepatic disease. No family antecedents were significant; 40.9% had dyslipidemia, 22.7% arterial hypertension, 22.7% chronic obstructive pulmonary disease (COPD), 13.6% hyperuricemia, 9.1% hypothyroidism, 4.5% diabetes mellitus type 2, and 4.5% carbohydrate intolerance; 40.9% had a body mass index > 30, and 27.3% presented gynecomastia/lipomastia. The region most frequently affected by fatty deposits was the neck. Madelung's disease affects mainly alcoholic males in their fourth decade of life. Hepatic diseases appear in most patients. Also associated with MD are high lipid blood levels, arterial hypertension, COPD, hyperuricemia, and obesity. MD type 1 is the most frequent phenotype and the neck the most common location for fatty masses. Recurrence after surgery, in the same location or different locations, is a frequent event, even in patients who later abstain from alcohol intake. This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors at <ExternalRef> <RefSource>www.springer.com/00266</RefSource> <RefTarget Address="http://www.springer.com/00266" TargetType="URL"/> </ExternalRef>.