Lipomatosis

from Wikipedia, the free encyclopedia
Classification according to ICD-10
E88.2 Lipomatosis, not elsewhere classified
ICD-10 online (WHO version 2019)

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

Synonyms and variants of lipomatosis

  • Symmetrical adenolipomatosis
  • Lipomatosis symmetrica
  • Diffuse symmetrical lipomatosis; generalized symmetrical lipomatosis; multiple symmetrical lipomatosis; circumscribed symmetrical lipomatosis
  • Lipomatosis simplex indolens
  • Launois-Bensaude syndrome, also known as Bensaude syndrome
  • Madelung disease (Madelung fat throat)

history

Lipomatosis was described by the two French doctors Pierre-Emile Launois (1856–1914) and Raoul Bensaude (1866–1938) in 1898. The German surgeon Otto Wilhelm Madelung described diffuse symmetrical lipomatosis with a preference for the neck area (Madelung fatty neck) .

classification

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

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

pathology

The causes of lipomatosis have not been precisely clarified, but latent familial accumulation and associations with various metabolic disorders such as hyperlipoproteinemia , diabetes mellitus , alcoholism , hyperuricaemia and hypothyroidism are discussed . Lipomatosis shows a common association with internal diseases such as polyneuropathy , lung carcinoma , Kaposi's sarcoma , myalgia, chronic liver damage, gynecomastia , arthralgia and varicose veins . Furthermore, autonomous growth of fat cells due to resistance to catecholamines is assumed.

histology

There is a diffuse, symmetrical, massive, doughy, coarse increase in the fatty tissue. Since there is no capsule, it is difficult to delineate the lipomas. Microscopically, a diffuse, non-septate proliferation of mature univacuolar lipocytes with tongue-shaped extensions into adjacent structures can be seen.

localization

Madelung disease patient

Madelung fat neck

Here, symmetrical, poorly demarcated lipomas appear predominantly on the neck and shoulder region . The mobility of the neck is limited. The disease occurs between the ages of 30 and 60. Men with a positive alcohol and liver history are particularly affected. Patients often experience dyspnoea from compression of the larynx and trachea. The Madelung fatty throat is often associated with neuropathies and autosomal dominant inheritance has been observed.

Further localization

The so-called buffalo hump can appear on the neck, while lipomatosis in the upper arm area shows up as puff-arm lipomatosis, and the flexor thighs can occasionally be affected.

clinic

The patients show a pseudo-athletic habitus. In type III, the fat deposits are predominantly found on the belly and in the pelvic girdle area. Lipomatosis can rarely only be located on the soles of the feet. This shows rapid, intermittent development and then stationary adipose tissue hyperplasia without a spontaneous regression or degeneration tendency.

therapy

No causal therapy is known to date. Due to the autonomy of adipose tissue, diets have little effect on the affected areas. Surgical reduction or liposuction are possible in principle, but tend to have a high recurrence rate. Success with salbutamol has been reported in some patients . The treatment of associated diseases (see above) often shows no effects on the adipose tissue hyperplasia.

forecast

With absolute abstinence from alcohol, the disease can come to a standstill. The adipose tissue hyperplasia cannot be influenced from a dietary point of view and shows resistance to tumor cachexia .

literature

  • Peter Altmeyer: therapy lexicon dermatology and allergology: therapy compact from A – Z. Springer, 2005, ISBN 3-540-23781-X , pp. 519ff.

Individual evidence

  1. ↑ Pierre-Emile Launois on whonamedit.com .
  2. ↑ Raoul Bensaude on whonamedit.com .
  3. ↑ Donhauser et al.: Benign symmetrical lipomatosis Launois-Bensaude type III and Bureau-Barrière syndrome. In: dermatologist. 1991; 42, pp. 311-314.
  4. ^ Peter Fritsch: Dermatology, Venereology: Basics, Clinic, Atlas. Springer, 2004, ISBN 3-540-00332-0 , p. 669.

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