Skeletal Radiology

, Volume 41, Issue 4, pp 489–490

Growing fatty mass in the back: diagnosis of a multiple symmetric lipomatosis (Madelung’s disease) in association with chronic alcoholism

  • Andreas Gutzeit
  • Christoph A. Binkert
  • Sina Schmidt
  • Abdul R. Jandali
  • Jochen Mutschler
  • Klaus Hergan
  • Sebastian Kos
Test Yourself: Answer

DOI: 10.1007/s00256-011-1280-6

Cite this article as:
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

Diagnosis

Multiple symmetric lipomatosis (Madelung’s disease)

Discussion

Multiple symmetric lipomatosis was first described in 1888 by the German surgeon Otto Madelung [1]. 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 [2].

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 [3].

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 [6].

There are only a few reports of complications. One report describes sudden deaths in association with Madelung’s diease [7]. There is also one description of spontaneous transformation of Madelung’s disease into a liposarcoma [8]. From the radiological point of view, this can be problematic because of difficulties in distinguishing between lipoma and well-differentiated liposarcoma [9].

To our knowledge, there is no consensus on disease management or therapy. Reducing alcohol consumption is recommended [10]. Surgery or lipid-lowering therapy may be recommended in the presence of symptomatic lipomas [11].

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 [14].

Acknowledgements

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.

Copyright information

© ISS 2011

Authors and Affiliations

  • Andreas Gutzeit
    • 1
    • 2
  • Christoph A. Binkert
    • 1
  • Sina Schmidt
    • 3
  • Abdul R. Jandali
    • 3
  • Jochen Mutschler
    • 4
  • Klaus Hergan
    • 2
  • Sebastian Kos
    • 5
  1. 1.Department of RadiologyCantonal Hospital WinterthurWinterthurSwitzerland
  2. 2.Department of RadiologyParacelsus Medical University SalzburgSalzburgAustria
  3. 3.Departement of SurgeryCantonal Hospital WinterthurWinterthurSwitzerland
  4. 4.Department of General and Social PsychiatryPsychiatric University Hospital ZurichZürichSwitzerland
  5. 5.Institute of Radiology and Nuclear MedicineUniversity Hospital BaselBaselSwitzerland

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