Lipomatosis

The lipomatosis (Latin: lipomatosis ) is a diffuse increase of adipose tissue ( Fettgewebshyperplasie ) at certain points of the body in humans and animals. Thus, the upper trunk, the hips and the inside of the body affected ( for example, the heart in the lipomatosis cordis ). To be distinguished is the lipomatosis dolorosa ( Dercum'sche disease), it is a particularly occurring in menopausal women disease that is characterized by the appearance of numerous small painful adipose tissue tumors.

• 5.1 Madelung's neck fat
• 5.2 Further localization

Synonyms and variations of lipomatosis

• Symmetrical Adenolipomatose
• Lipomatosis symmetrica
• Diffuse symmetrical lipomatosis; generalized symmetric lipomatosis; Multiple symmetric lipomatosis; circumscribed symmetrical lipomatosis
• Lipomatosis simplex indolens
• Launois - Bensaude syndrome called, as well as Bensaude syndrome
• Madelung 's disease ( Madelung fat neck)

History

The lipomatosis was described by the two French physicians Pierre -Emile Launois (1856-1914) and Raoul Bensaude (1866-1938) in 1898., The German surgeon Otto Wilhelm Madelung described the diffuse symmetrical lipomatosis with preference of the neck region ( Madelung fat neck).

Classification

The classification on the basis of the distribution pattern of adipose tissue (after Donhauser ):

• Type I: neck and shoulder type ( Madelung's neck fat, localized type)
• Type II: shoulder girdle type ( pseudo- athletic type )
• Type III: pelvic girdle type ( gynäkoider type)
• Type IV: Abdominal type.

Pathology

The causes of the lipomatosis are not clearly understood, are discussed latent familial clustering and associations with various metabolic disorders such as hyperlipidemia, diabetes mellitus, alcoholism, hyperuricemia and hypothyroidism. The lipomatosis is a frequent association with internal diseases, such as polyneuropathy, lung cancer, Kaposi 's sarcoma, myalgia, chronic liver damage, gynecomastia, arthralgia and varicose veins. Furthermore, autonomous growth of fat cells due to resistance of catecholamines is assumed.

Histology

It shows a diffuse symmetric, massive, doughy rough proliferation of adipose tissue. Since no capsule is present, the demarcation of lipomas is difficult. Microscopically shows a diffuse, non-septate proliferation of mature lipocytes univakuolärer with tongue-shaped foothills into adjacent structures.

Localization

Madelung's neck fat

Here arise predominantly at the neck and shoulder region symmetric ill-defined lipoma a. The mobility of the neck is limited. The disease occurs between 30 and 60 years. There are primarily affected men with a positive alcohol and liver history. Patients often occurs dyspnea by laryngeal and Tracheakompression. The Madelung - fat neck is often associated with neuropathies and autosomal dominant patterns of inheritance have been observed ..

Further localization

At the neck of the so-called buffalo hump can occur, however, shows up in the upper arm area, the lipomatosis as Puffärmellipomatose, the thigh flexor surfaces can occasionally be affected.

Clinic

Patients have a pseudo athletic habitus. In type III, the fat deposits are mainly fat apron -like on the abdomen and the pelvic girdle. Rarely, the lipomatosis be localized exclusively to the soles of the feet. This shows a rapid, batch-wise development and then stationary Fettgewebshyperplasie without spontaneous involution or degeneration trend.

Therapy

So far, no causal therapy is known. By autonomy of adipose tissue diets point to the affected areas only little effect. Operative reduction or liposuction are possible in principle, but tend to be high recurrence rate. In some patients, success has been reported with salbutamol. The treatment of associated diseases (see above) often shows no effect on the Fettgewebshyperplasien.

Forecast

With absolute abstinence from alcohol, it may stop the disease. The Fettgewebshyperplasie is dietary influence and are showing a resistance in cancer cachexia.