Madelung Disease is one of the rarely occurring health conditions when growth of lipomas or fatty tumors takes place around the upper trunk, upper arms, shoulders and neck. These look very odd and the affected person gets irregular shape in those parts of the body which catches immediate attention of outsiders and cause peripheral neuropathy. Madelung Disease is also known as multiple symmetric lipomatosis, benign symmetrical lipomatosis, and Launois-Bensaude.

German surgeon, Otto Wilhelm Madelung described this disease and its symptoms in details for the first time and since then the disease is mostly called ‘Madelung Disease’. Although research suggests a strong connection between alcohol consumption and Madelung disease or benign symmetric lipomatosis, yet, in some situations, it is found that non-alcoholics are also affected by Madelung disease. The signs and symptoms of Madelung disease or benign symmetric lipomatosis vary from person to person. If not treated in time, the fatty tissues accumulate quickly hampering the normal mobility of the neck or the other affected body parts. Mild to severe pains may grow disturbing the normal activities of the affected people. The lipomas don’t become cancerous normally, but may be associated with mutations in mitochondrial DNA.

Madelung Disease or Benign Symmetric Lipomatosis

Symptoms of Madelung Disease or Benign Symmetric Lipomatosis

The signs and symptoms of Madelung disease or benign symmetric lipomatosis vary from one person to another. It is characterized by the symmetric growth of fatty tumors around the neck, shoulder, upper arms and upper trunk. Again, these fatty growths may be fast in some persons and very slow in others. In most of the cases the lipomas are benign, but may cause pain if early treatment is not started.

Multiples of symptoms are associated with Madelung disease or benign symmetric lipomatosis. Some of these are as follows –

  • Limitations in joint mobility
  • Arthralgia
  • Gait disturbance
  • Paresthesia
  • Hyperglycemia
  • Resistance to insulin
  • Hepatomegaly.

Patients with Madelung disease or benign symmetric lipomatosis may sometimes also develop peripheral neuropathy including other neurological disturbances like difficulty in swallowing, sleep problems, hoarseness, hypertension, irregular breathing and increase in heart rate. In advanced stages, it may lead to liver issues as well as hypothyroidism.

Epidemiology of Madelung Disease or Benign Symmetric Lipomatosis

As per the statistics, people between the ages of 30 to 70 years with a history of Mediterranean ancestry are found to be vulnerable to benign symmetric lipomatosis. People with higher proneness of alcohol intake are also at higher risk of having this rare disease. The disease is not much visible, 1 in 25000 people have been detected with some symptoms of Madelung disease or benign symmetric lipomatosis. Moreover, males are more affected by Madelung disease than females with a ratio of 30:1.

Prognosis of Madelung Disease or Benign Symmetric Lipomatosis

In 60% of cases, there have been detected recurrences of Madelung disease or benign symmetric lipomatosis leading to further surgeries or liposuction. However, risk of recurrence of Madelung disease or benign symmetric lipomatosis reduces drastically when treatment procedures and behavioral changes are followed strictly.

Causes of Madelung Disease or Benign Symmetric Lipomatosis

The precise basic cause of Madelung disease or benign symmetric lipomatosis is not clear to the researchers till now. However, at different points of time several theories have been proposed by the researchers which are as follows:

  • Body's incapacity to metabolize consumed fat properly, which is an endocrine disorder, may be a cause of Madelung Disease or benign symmetric lipomatosis.
  • Defects in enzyme secretion that prevents assimilation of fat in the circulatory system may be another reason of Madelung disease or benign symmetric lipomatosis.
  • An alteration in the external surface of cells could foil the breakdown of fat leading to the distinctive signs and symptoms of Madelung disease or benign symmetric lipomatosis.
  • Researchers have also found a relation between alcohol consumption with the development of Madelung disease or benign symmetric lipomatosis. It is found that in 90% of cases, the patients were alcoholic.
  • Though no direct evidence of genetic link is established, researchers have found that in some cases some changes in mitochondrial DNA have taken place. These changes may create the same problem in the predecessors if other risk factors also exist simultaneously.

Risk Factors of Madelung Disease or Benign Symmetric Lipomatosis

It is tough to list up all the risk factors, since researchers are yet to accumulate sufficient evidence against all the risk factors of this rare disease. However, people predisposed to alcohol abuse and having history of Madelung disease in family are at higher risk of getting affected by Benign Symmetric Lipomatosis.

Complications Related to Madelung Disease or Benign Symmetric Lipomatosis

In extremely rare conditions, lipomas in Madelung Disease or benign symmetric lipomatosis may become malignant. A few cases of head and neck cancers are detected with Madelung Disease; however, researchers are yet to associate any precise link of Madelung disease or benign symmetric lipomatosis with cancer. The death rate of 10% in case of delayed diagnosis is established and in this case patients have died of asphyxiation. Thus, except recurrence of lipomas no other serious complications are normally found in Madelung Disease or Benign Symmetric Lipomatosis.

Diagnosis of Madelung Disease or Benign Symmetric Lipomatosis

CT scan and MRI are used to detect Madelung Disease or benign symmetric lipomatosis; however experts also take lifestyle and family history of the patients before diagnosing madelung disease or benign symmetric lipomatosis. In some cases, doctors also ask for biopsies of the lipomas to confirm the disease.

Treatment of Madelung Disease or Benign Symmetric Lipomatosis

In most of the cases, lipomas are surgically operated, but in recent times liposuction is mostly used to remove the lipomas quickly and effectively. Liposuction method is technically perfect and requires minimum incision; hence, this method leaves small scars in the operated areas. Moreover, liposuction has no risk and side-effects, which is easy to adopt and patients recover quicker than normal surgical methods. However, in some critical situations like presence of large deposition of fats with superficial hardness, and/or lipomas with airway compressions etc., are times where traditional surgery becomes inevitable.

Surgical excision extirpates the lipomas more effectively, but it is not always possible to remove the lipomas completely through surgical process and liposuction. As a result, there remains a keen possibility of recurrence of same symptoms if proper treatment procedure is not followed and along with lifestyle changes.

Medications like salbutamol helps to breakdown the fatty acids in the body, thus many doctors apply it accordingly. At the same time, abstaining from alcohol, getting strong regime for weight loss and treatment for endocrine abnormalities are required to prevent further growth of lipomas.

Conclusion

Madelung disease or benign symmetric lipomatosis is categorized as a rare one when mostly benign lipomas are developed in certain parts of the body. Much of the disease and its causes are still not clear to the researchers, but a few risk factors like alcoholism and family history are found to be closely linked with the disease. The lipomas look very odd; hence, doctors advise for the earliest possible liposuctions or surgeries, depending on the severity of each case. A certain lifestyle changes and some medications are needed even after the surgery, to prevent recurrence of the disease.

Written, Edited or Reviewed By:

, MD, FFARCSI

Last Modified On: June 22, 2016

Pain Assist Inc.

Pramod Kerkar
  Note: Information provided is not a substitute for physician, hospital or any form of medical care. Examination and Investigation is necessary for correct diagnosis.

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