Lipomatosis

Introduction

The term lipomatosis describes a diffusely distributed, unnatural increase in fatty tissue affecting different parts of the body. Lipomatosis (Greek: lipos = fat; -om = tumor-like tumor; -ose = chronic progressive disease) is a term used to describe several clinical pictures, some of which cannot be completely separated from one another, but all of which have in common increased fatty tissue in the form of tumors. It is a rare metabolic disease with a mechanism that is still insufficiently understood.

Lipomatosis can affect various regions of the body such as the head, neck, thighs and upper arms, abdomen and back. A form is also known in which the internal fatty tissue of organs such as the pancreas is increased, in some cases the spinal canal is affected. Lipomatosis is primarily not a malignant disease, but rather a pathological, but benign new formation of fatty tissue (fatty tissue hyperplasia) and creates above all cosmetic suffering.

However, accumulation between or within organs can be associated with increased risk of disease. Various forms of lipomatosis are known. The most common and best known type is symmetrical (adeno-)lipomatosis, which is also known as Launois-Bensaude syndrome after its first descriptors.

A special form, in which the head and neck are mainly affected, is called Madelung-Lipomatose. A classification distinguishes four types: Furthermore, the disease is named after the affected area, such as Lipomatosis cordis (cor=the heart), i.e. an increase in fat on the heart. A disease that occurs in some women during menopause, the lipomatosis dolorosa, is also called lipomatosis, but has other causes and mechanisms and is not discussed in this article.

  • Type I: Neck and neck type (Madelung fat neck, localized type)
  • Type II: shoulder girdle type (pseudoathletic type)
  • Type III: Pelvic girdle type (gynecological type)
  • Type IV: Abdominal type

Causes

The causes of lipomatosis are still the subject of intensive research. Nevertheless, the processes behind the development of this disease pattern are not well understood. In some patients there is an accumulation within the family, so that a genetic component is assumed.

Furthermore, it has been observed that patients with lipomatosis often have additional metabolic disorders that are probably related to the occurrence of lipomatosis. For example, there seems to be an association with diabetes mellitus, an underfunction of the thyroid gland (hypothyroidism) or other lipid metabolism diseases. Many studies associate lipomatosis with long-term excessive alcohol consumption.

It has been found that men are affected about 13 times more frequently than women, especially when it comes to people who abuse alcohol. At the cellular level, the theory that the growing and multiplying fat cells no longer respond to the body’s own signals is widely accepted. Hormones such as adrenaline or noradrenaline can therefore no longer exert their effect on the cell, which therefore grows autonomously. This would explain why, even in extremely thin patients such as tumor patients, lipomas persist even though the remaining fatty tissue has largely been broken down. Another specific cause is the treatment of HIV with a certain drug, which in up to 40% of cases leads to lipomatosis as a side effect.