Madelung's disease is a disorder of fat metabolism (lipid storage) that results in an unusual accumulation of fat deposits around the neck and shoulder areas. Adult alcoholic males are most often affected, although women and those who do not drink alcohol can also get Madelung's disease.
Madelung’s disease is characterized by massive deposits of fatty tumors (lipoma) located symmetrically around the neck, and on the shoulders, upper arms and upper trunk. These abnormal fat deposits may grow rapidly over the course of months or more slowly over a period of years. The rest of the body may be lean in contrast to the affected parts. The fatty deposits do not indicate malignancy and, while disfiguring, may not impair functioning.
Peripheral neuropathy, or impaired function of the nerves in the arms and legs, often accompanies Madelung’s disease, especially as the affected person grows older. However, these neurological deficits may be difficult to distinguish from the long-term effects of alcoholism when overuse of alcohol is a factor.
Sometimes, there are metabolic abnormalities and other diseases associated with Madelung’s disease. These may include diabetes mellitus, hypertension, and liver disease.
The exact cause of Madelung’s disease is not known. The body’s inability to properly metabolize fat indicates that it may be an endocrine disorder. Some scientists believe a predisposition to the disorder may be inherited and there have been some reports of cases that appear to be familial. However, the mode of transmission in those cases has not been determined.
Madelung’s disease most frequently affects middle-aged males. The condition is most common in those who abuse alcohol. However, this disease is also found in women and persons who do not consume alcohol. For reasons that are unclear, the disorder appears to be more prevalent in Europe than in the United States.
The diagnosis of Madelung's disease may involve ultrasound images, CAT scans, or magnetic resonance imaging.
Treatment consists of surgical removal of the fatty deposits from the areas around the head, neck, shoulders and trunk. Liposuction has been used successfully to remove single fatty tumors.
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