Disease Information for Multiple Symmetric lipomatosis

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Clinical Manifestations
Signs & Symptoms
Bull neck/swelling
Lumpy deforming swellings neck,chin,shoulders
Upper extremity distribution/nodules
Formication/Crawling insects sensation
Hot Flushes
Painful subcutaneous nodules
Skin nodules/Subcutaneous nodules
Subcutaneous nodules
Subcutaneous Nodules in Elderly
Subcutaneous/mass
Supraclavicular Fat Pads Deposit
Dysesthesias
Hot flashes
Malaise
Otherwise well Isolated problem
Weight gain
Demographics & Risk Factors
Established Disease Population
Patient/Alcoholism/chronic alcoholic
Patient/Excess alcohol habit
Population Group
Man
Middle Age Adult
Sex & Age Groups
Population/Fifties adult
Population/Male
Population/Man patient
Population/Middle-aged adult
Associated Diseases & Rule outs
Associated Disease & Complications
Familal Multiple Lipomatosis
Lipoma
Multiple symmetric lipomatosis
Disease Mechanism & Classification
Class
CLASS/Primary organ/system disorder (ex)
CLASS/Soft Tissue Involvement
CLASS/Neck disorder (ex)
CLASS/Adipose tissue disorder (ex).
CLASS/Dermatologic/Subcutaneous (category)
CLASS/Subcutaneous/Panniculus tissue disorder
CLASS/Shoulder involvement/disorder (ex)
Pathophysiology
Pathophysiology/Subcutaneous manifestation/involvement
Process
PROCESS/Idiopathic/unclassified/unknown (category)
Synonyms
Synonym
Lipomatoses Multiple Symmetrical, Lipomatosis Multiple Symmetrical, LMS Multi symmet lipomatosis, LMS Multiple symmetrical lipomatosis, Mult symmetrical lipomatosis, Multiple Symmetrical Lipomatoses, Multiple Symmetrical Lipomatosis, Multiple symmetrical lipomatosis (disorder), Symmetrical Lipomatoses Multiple, Symmetrical Lipomatosis Multiple, Synonym/Benign Symmetrical Lipomatosis, Synonym/Familial Multiple Lipomatosis variant, Synonym/Launois-Bensaude syndrome, Synonym/Madelung disease, Synonym/Madelung's disease (Variant), Synonym/MSL Syndrome
Treatment
Drug Therapy - Indication
RX/No effective/definitive treatment yet available
Definition

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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NEJM (The New England Journal of Medicine)
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