Growing fatty mass in the back: diagnosis of a multiple symmetric lipomatosis (Madelung’s disease) in association with chronic alcoholism
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- Gutzeit, A., Binkert, C.A., Schmidt, S. et al. Skeletal Radiol (2012) 41: 489. doi:10.1007/s00256-011-1280-6
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Part 2: answer
Growing fatty mass in the back: diagnosis and discussion
Multiple symmetric lipomatosis (Madelung’s disease)
Multiple symmetric lipomatosis was first described in 1888 by the German surgeon Otto Madelung . Several years later, Lanois and Bensaude described a further case series of the same entity, which is now refered to as Madelung’s disease or Lanois-Bensaude syndrome .
Most affected patients are men with a history of chronic alcoholism. Typical signs of this condition are slowly growing fatty masses around the neck, upper part of the arms, pelvis, back and the thigh. There is a predilection for people from the Mediterranean .
The pathogenesis of this syndrome is unknown. Recently published data suggest that children with similar findings might have an underlying mitochondrial dysfunction [4, 5]. However the great majority of cases of Madelung’s disease occur in men aged between 30 and 70 years with known alcoholism .
There are only a few reports of complications. One report describes sudden deaths in association with Madelung’s diease . There is also one description of spontaneous transformation of Madelung’s disease into a liposarcoma . From the radiological point of view, this can be problematic because of difficulties in distinguishing between lipoma and well-differentiated liposarcoma .
To our knowledge, there is no consensus on disease management or therapy. Reducing alcohol consumption is recommended . Surgery or lipid-lowering therapy may be recommended in the presence of symptomatic lipomas .
The differential diagnosis might include dietary obesity, corticosteroid use, hamartomatous polyposis and adiposis dolorosa (Dercum’s disease). Our patient had a body mass index of 18.7 (normal) and had no history of corticosteroid use. Multiple subcutaneous lipomas may occur in Bannayan-Zonana syndrome, another of the hamartomatous polyposis syndromes, in which case it may be associated with a disturbed lipid distribution and storage [12, 13]. Adiposis dolorosa also results in multiple lipomas, but these are typically described as painful .
We want to thank the Department of Pathology of Cantonal Hospital Winterthur, especially Dr. Ivo Tosony, for helping us in the preparation of the histological image.