Disease Information for Multiple Symmetric lipomatosis

Madelung"s Disease; Benign Symmetrical Lipomatosis;

Launois-Bensaude; MSL; Multiple Symmetric Lipomatosis

Madelung"s disease is a disorder of fat metabolism (lipid storage) that results in an unusual accumulation of fat deposits around the neck and shoulder areas; Adult alcoholic males are most often affected, although women and those who do not drink alcohol can also get Madelung"s disease;[nord 2005]------------------------------

Madelung"s disease or Benign Symmetric Lipomatosis is a "sight diagnosis"; Anyone who has seen it will recognize it immediately while those who haven"t probably won"t;

A professional woman in her late 50"s came to me for a third opinion regarding a peculiar, progressive and disfiguring deposition of fat around her neck and over her shoulders; Her hips and legs remained normal in appearance as did her face but, gradually over a period of two years, she began to look like she was wearing shoulder pads;

She also complained of weakness and on exam she had symmetrical weakness of hip and shoulder girdle muscles with normal peripheral strength; She had a selective loss of vibratory sensation in her feet with preserved ankle reflexes; and difficulty with tandem gait; She gave a history of years "1-2 litres of wine per day;" She had enlarged red cells with an MCV (mean corpuscular volume) of 106; mildly elevated liver enzymes and a liver ultrasound showing hyperechoicity consistent with fatty infiltration;

An EMG showed mild myopathic findings in the deltoids and the feeling of the needles suggested the hypertrophic tissue was fat, not muscle; A biopsy that probably included some sternocleidomastoid showed subsarcolemmal masses consistent with alcoholic myopathy; MADELUNG"S DISEASE :According to Philip W. Allen in Tumors and Proliferations of Adipose Tissue, Masson Publishing, 1981: Madelung"s disease "is almost exclusively a disease of middle aged alcoholic males, although females and non-alcoholics are sometimes said to be affected; A typical patient is likely to be a male aged about 50 years of age who complains of a socially embarassing or grotesque enlargement in the neck and chin; the appearance of the masses is preceded by 10 or more years of heavy drinking; generalized obesity is not a feature"

The differential includes obesity, steroid adiposity, lipomas and liposarcoma; Allen states that the only serious danger is possible involvement of the larynx causing respiratory obstruction; The prognosis "is probably more dpendent on the associated alcoholism"

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