Madelung's Disease

Madelung's Disease

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Madelung's Disease is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • Benign Symmetrical Lipomatosis
  • Launois-Bensaude
  • MSL
  • Multiple Symmetric Lipomatosis

Disorder Subdivisions

  • None

General Discussion

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.

Symptoms

Madelung's disease is characterized by massive deposits of fatty tumors (lipoma) located symmetrically around the neck, and on the shoulders, upper arms and upper trunk. These abnormal fat deposits may grow rapidly over the course of months or more slowly over a period of years. The rest of the body may be lean in contrast to the affected parts. The fatty deposits do not indicate malignancy and, while disfiguring, may not impair functioning.



Peripheral neuropathy, or impaired function of the nerves in the arms and legs, often accompanies Madelung's disease, especially as the affected person grows older. However, these neurological deficits may be difficult to distinguish from the long-term effects of alcoholism when overuse of alcohol is a factor.



Sometimes, there are metabolic abnormalities and other diseases associated with Madelung's disease. These may include diabetes mellitus, hypertension, and liver disease.

Causes

The exact cause of Madelung's disease is not known. The body's inability to properly metabolize fat indicates that it may be an endocrine disorder. Some scientists believe a predisposition to the disorder may be inherited and there have been some reports of cases that appear to be familial. However, the mode of transmission in those cases has not been determined.

Affected Populations

Madelung's disease most frequently affects middle-aged males. The condition is most common in those who abuse alcohol. However, this disease is also found in women and persons who do not consume alcohol. For reasons that are unclear, the disorder appears to be more prevalent in Europe than in the United States.

Standard Therapies

Diagnosis

The diagnosis of Madelung's disease may involve ultrasound images, CAT scans, or magnetic resonance imaging.



Treatment

Treatment consists of surgical removal of the fatty deposits from the areas around the head, neck, shoulders and trunk. Liposuction has been used successfully to remove single fatty tumors.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government website.



For information about clinical trials being conducted at the National Institutes of Health (NIH) Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:



Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com

References

TEXTBOOKS

Foster DW, The lipodystrophies and other rare disorders of adipose tissue. In: Fauci AS et al., eds. Harrison's Principles of Internal Medicine. New York, NY. McGraw-Hill Companies, Inc; 1998;2212.



REVIEW ARTICLES

Lee HW, Kim TH, Cho JW, et al. Multiple symmetric lipomatosis: Korean experience. Dermatol Surg. 2003;29:235-40.



Cossarizza A, Mussini C, Vigano A. Mitochondria in the pathogenesis of lipodystrophy induced by anti-HIV antiretroviral drugs: actors or bystanders? Bioessays. 2001;23:1070-80.



Kakuda TN. Pharmacology of nucleoside and nucleotide reverse transcriptase inhibitor-induced mitochondrial toxicity. Clin Ther. 2000;22:685-708.



JOURNAL ARTICLES

Gonzalez-Garcia R, Rodriguez-Campos FJ, Sastre-Perez J, et al. Benign Symmetric Lipomatosis (Madelung's Disease): Case reports and Current Management. Aesthetic Plast Surg. 2004 May 28[Epub ahead of print]



Verhelle NA, Nizet JL, Van den Hof B, et al. Liposuction in benign symmetric lipomatosis: sense or senseless? Aesthetic Plast Surg. 2003;27:319-21.



Busetto L, Strater D, Enzi G, et al. Differential clinical expression of multiple symmetric lipomatosis in men and women. Int J Obes Relat Metab Disord. 2003;27:1419-22.



Morelli F, De Benedetto A, Toto P, et al. Alcoholism as a trigger of multiple symmetric lipomatosis? J Eur Acad Dermatol Venereol. 2003;17:367-69.



Enzi G, Busetto L, Ceschin E, et al. Multiple symmetric lipomatosis: clinical aspects and outcomes in a long-term longitudinal study. Int J Obes Relat Metab Disord. 2002;26:253-61.



Ujpal M, Nemeth ZS, Reichwein A, et al. Long-term results following surgical treatment of benign symmetric lipomatosis (BSL). Int J Oral Maxillofac Surg. 2001;30:479-83.



FROM THE INTERNET

McKusick VA, ed. Online Mendelian Inheritance In Man (OMIM). The Johns Hopkins University. Lipomatosis, Familial Benign Cervical. Entry Number; 151800: Last Edit Date; 7/7/1997.



Madelungs Disease: Inherited from an Ancient Mediterranean Population. 1999.

http://content.nejm.org/cgi/content/short/340/19/1481

Resources

NIH/National Institute of Diabetes, Digestive & Kidney Diseases

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Tel: (301)496-3583

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Internet: http://www2.niddk.nih.gov/



About Madelungs

7251 Brentwood Blvd, Apt. 163

Brentwood, CA 94513

USA

Tel: (925)516-6744

Email: madelungs@yahoo.com

Internet: http://www.aboutmadelungs.net/



Genetic and Rare Diseases (GARD) Information Center

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Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

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