Friday, September 14, 2007

Madelung's Disease: lipomatosis

Madelung's Disease: Inherited from an Ancient Mediterranean Population?
1: N Engl J Med. 1999 May 13;340(19):1481.Click here to read


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A 44-year-old man was evaluated for symmetric deposits of fat on his arms and legs. At the age of 26 years, he had been hospitalized because of axonal polyneuropathy of the legs and alcoholic fatty liver. Subsequently, fat deposits characteristic of multiple symmetric lipomatosis slowly developed (Panel A). In addition to the fat deposits, the patient had macrocytic anemia, peripheral neuropathy, and alcoholic fatty liver. Glucose and lipid metabolism was normal. No other family members had a similar distribution of fat.

Multiple symmetric lipomatosis, also known as Madelung's disease or the Launois–Bensaude syndrome, is characterized by painless, diffuse, subcutaneous deposits of adipose tissue in the neck, upper trunk, and arms and legs. It is frequently associated with alcoholic liver disease, macrocytic anemia, and peripheral neuropathy, as in this patient. It is a rare inherited condition of unknown cause that affects mainly men, with a higher incidence in the Mediterranean area. The distribution of fat resembles that of a statue known as the Capestrano warrior (Panel B), which was found at an archaeological site in the Abruzzi region of Italy and dates to the sixth century B.C. The imposing figure, about 250 cm tall, with its unique somatic aspect, characterized by big hips and wide shoulders, strongly resembles a patient with Madelung's disease. This similarity could be coincidental, but it could also represent a genetic relation.



Claudio Feliciani, M.D.
UniversitĂ  G. D'Annunzio
66013 Chieti, Italy


Pierluigi Amerio, M.D.
UniversitĂ  Cattolica
00100 Rome, Italy

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