Multiple Symmetric Lipomatosis: Fat in Bizarre Places. A Pathognomonic Sign?

Program: Abstracts - Orals, Featured Poster Presentations, and Posters
Session: SUN 723-739-Lipids: Therapeutics & Case Reports
Clinical
Sunday, June 16, 2013: 1:45 PM-3:45 PM
Expo Halls ABC (Moscone Center)

Poster Board SUN-737
Shruti Mahendra Gandhi*1, Kenneth L Becker1, Eric S Nylen2 and Michael Scott Irwig1
1George Washington University, Washington, DC, 2VAMC, Washington, DC
Introduction: Multiple Symmetric Lipomatosis (aka Madelung’s Disease or Launois Bensaude Syndrome) is a rare disorder of unknown etiology that is characterized by a painless, symmetrical accumulation of abnormal subcutaneous adipose tissue. The majority of those affected are middle aged men of Mediterranean origin with chronic alcohol use. There is no known marker for this condition, making physical findings essential to the diagnosis. We have assembled a series of 12 patients in our clinic with this syndrome.

Index Case: A 53 year old postmenopausal female of Scottish descent was referred for abnormal adipose accumulation. Over one year, she rapidly developed soft tissue masses progressing from the base of her neck to her shoulders and upper arms. She subsequently was found to have decreased muscle strength, requiring assistance of handrails and inability to do sit-ups. She limited her time in public due to her appearance. The social history was notable for consumption of two glasses of wine per night for twenty years.

Physical exam was notable for extensive fat accumulation on the neck, shoulders, arms and upper back. The fat was fixed and non-tender. Lab and Imaging results were significant for transaminase elevation and hyperlipidemia. Abdominal ultrasound showed no evidence of fatty liver. The CT of the neck showed fat extending from the posterior cervical spine musculature to the upper chest. It was isodense to subcutaneous fat without clear separation.

Upon review of the 11 other male cases, each patient presented in a similar fashion after years of alcohol use. They had symmetric excess subcutaneous fat tissue localized to posterior neck, posterior cervical and upper back, and abdomen. Interestingly, half of these patients were noted to have bilateral discrete fat localized in the popliteal fossa.  

Conclusion: The index case describes a typical presentation of Multiple Symmetric Lipomatosis. It is also somewhat unique as the patient is a Non Mediterranean female (normally 15:1 male to female ratio). There have been three types of lipomatosis described based on the predominant location of fat deposition. Type 1 is fat localized to head/neck; type 2, fat is localized to the shoulder girdle, upper arms, thorax, back, abdomen and upper buttocks; and type 3 have fat in the upper thigh. Considering that half of our patients had fat in the popliteal fossa, this bizarre location could serve as a new pathognomonic physical finding with diagnostic utility.

Nothing to Disclose: SMG, KLB, ESN, MSI

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