Images in Clinical Medicine

Madelung's Disease: Inherited from an Ancient Mediterranean Population?

Claudio Feliciani, M.D., and Pierluigi Amerio, M.D.

N Engl J Med 1999; 340:1481May 13, 1999

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Figure 1 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.
Università G. D'Annunzio, 66013 Chieti, Italy

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

Citing Articles (8)

Citing Articles

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   Stephen H. Caldwell, Curtis K. Argo, Abdullah M. S. Al-Osaimi. 2011. Nonalcoholic Fatty Liver Disease. , 868-907.
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   Stephen H. Caldwell, Curtis K. Argo. 2011. Non-Alcoholic Fatty Liver Disease and Nutrition. , 546-567.
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   Curtis K. Argo, Stephen H. Caldwell. (2009) Epidemiology and Natural History of Non-Alcoholic Steatohepatitis. Clinics in Liver Disease 13:4, 511-531
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   Kerrie Schoffer, Ian Grant. (2006) Multiple lipomas, alcoholism, and neuropathy: Madelung's disease or MERRF?. Muscle & Nerve 33:1, 142-146
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   F Morelli, C Feliciani, A De Benedetto, P Toto, A Tulli. (2003) Alcoholism as a trigger of multiple symmetric lipomatosis?. Journal of the European Academy of Dermatology and Venereology 17:3, 367-369
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   Alfredo Muoz-Mlaga, Juan Bautista, Jose A. Salazar, Isabel Aguilera, Raul Garcia, Isidoro Chinchon, M. Dolores Segura, Yolanda Campos, Joaquin Arenas. (2000) Lipomatosis, proximal myopathy, and the mitochondrial 8344 mutation. A lipid storage myopathy?. Muscle & Nerve 23:4, 538-542
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   James C. Wilson. (2000) Making disability visible: How disability studies might transform the medical and science writing classroom. Technical Communication Quarterly 9:2, 149-161
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   J. G. Heckmann, B. Kraus, H. Stefan, B. Neundorfer. (2000) Progressive muscle pain, weight gain, fatique and unusual body shape change. European Journal of Neurology 7:2, 243-244
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