Definition
Lipomatosis is a disorder that presents abnormal and circumscribed expansions of lipid tissue: it is in fact characterized by the formation of single or multiple lipomas, which can spread in all fatty areas of the body.
Lipomatosis is a widespread condition and the resulting problem is predominantly aesthetic. However, this depends strongly on the personal interpretation of the disorder, as well as on the type of lipomatosis that afflicts the patient. As you will see in the article, in fact, some forms of lipomatosis can cause very serious complications.
Features
As mentioned, lipomatosis is characterized by the formation of a large number of lipomas which tend to spread to different areas of the body.
Lipomas are mainly composed of adipose tissue (but in some cases can also involve other tissues), have a soft consistency, are mobile to the touch and generally asymptomatic. These accumulations of fat that occur in the form of subcutaneous reliefs are considered, in most cases, as benign tumors.
Despite this, the clinical picture that derives from lipomatosis could generate interpretative problems regarding the differential diagnosis. The lipomas, in fact, tend to infiltrate between the various tissues and are not prone to delimit themselves in specific areas: these factors contribute to the peculiarity of lipomatosis.
Classification
Classification and types of lipomatosis
Due to the fact that lipodystrophy is a phenomenon that affects every area of the body, we can distinguish some clear and defined clinical pictures, based on the area affected.
The main forms of lipomatosis with their characteristics will be briefly described below.
Symmetric multiple lipomatosis
Represents the most widespread form of lipomatosis, in which the lipomas are extensive and evolved, particularly in the region that affects the neck, nape, groin, thighs, deltoid and scapulae. Lipomatosis is called "symmetrical" to emphasize the symmetry with which lipomas develop. It is a lipomatosis that mainly affects humans, whose genetic transmission is autosomal dominant, but other characters could also favor the development of the disease (chronic alcoholism).
In this pathology two other sub-categories are distinguished:
- Type I symmetrical multiple lipomatosis, which mainly affects the neck and shoulder blades, the hollow veins and the trachea are the areas most affected and the lipomas present could be displaced in such a way as to attribute a taurine appearance to the affected subject.
- Type II symmetrical multiple lipomatosis, on the other hand, is characterized by very expanded and excessively evolved lipomas, such as to reproduce an obesity-like situation. Multiple symmetrical lipomatosis is often also associated with ulcerations (caused by decreased peripheral sensitivity) and neuropathy. The surgical removal of lipomas is the only possible remedy.
Pelvic lipomatosis
In this form of lipomatosis lipomas proliferate in the pelvic retroperitonal area. The disease has a higher incidence rate in the black race and among adult males who have passed the third decade. The causes that trigger pelvic lipomatosis are uncertain; however, some hypotheses have been formulated according to which the disease is related to localized obesity, but there is no clear evidence to prove this relationship. It does not seem a case, in fact, that the pelvic lipomatosis is found above all among the obese subjects; often, it involves secondary symptoms such as constipation, fever, pelvic pain, hydronephrosis (swelling of a kidney) and frequent urination.
Epidural lipomatosis
In this condition, the lipomas are located in the epidural adipose tissue. It occurs in obese subjects who have endogenous or iatrogenic hypercortisonemia (caused by drugs). Epidural lipomatosis can be asymptomatic or, in some cases, cause spinal pain and reduced muscle strength in the legs. In addition, epidural lipomatosis has been reported in some individuals with Cushing's syndrome.
Painful lipomatosis
Painful lipomatosis (or Dercum syndrome ) mainly affects women in senescence. The pathology manifests itself with multiple and painful lipomas.
Lipomatosiaddominaliomediastino-abdominal
This type of lipomatosis is often associated with diseases such as diabetes mellitus and disorders such as fluid effusion in the peritoneum and dyspnea (difficulty in breathing).
Krabbe lipomatosis
Krabbe's lipomatosis is a hereditary syndrome characterized by the development of subcutaneous fat lumps (lipomas), not very large but relatively annoying and painful. They are localized preferentially in the arms, thighs and trunk, areas in which sensitivity is altered.
Krabbe's lipomatosis is a genetic condition transmitted as an autosomal dominant trait.
Mesosomatic Roch-Leri lipomatosis
The mesosomatic lipomatosis of Roch-Leri belongs to the class of rare lipomatosis. Also in this case, it is an autosomal dominant condition, whose lipomas spread over the forearms, thighs and trunk. Individuals with Roch-Leri mesosomatic lipomatosis experience pain only when lipomas are crushed against a rigid surface and are related to vascular or muscular appendages. Generally, they do not evolve into malignant tumors.
Other forms of lipomatosis can be classified into:
- Asymmetric lipomatosis.
- Lipomatosis of the anal and perianal hilum.
- Pancreas lipomatosis .
- Lipomatosis of the bachelor girdle.
Causes
In general, the causes that favor lipomatosis are still being studied. However, in addition to the most accredited genetic predisposition, it seems that lipomatosis is related to neurological and hormonal alterations, which could contribute to the formation and subsequent growth of the lipoma.
Furthermore, there is a theory according to which lipomatosis - hence the formation of lipomas - is connected to causes of traumatic origin.
Finally - for some types of lipomatosis - there are conditions that can represent risk factors (or, in some cases, the triggering cause) for the formation of the disorder, such as alcohol abuse (alcoholism), the use of some types of drugs or obesity.
Symptoms and Complications
The symptoms of lipomatosis may be different depending on the area in which it occurs and depending on its extension. Therefore, the symptomatology of this disorder depends on the form of lipomatosis that affects the patient.
Generally speaking, lipomas that give rise to lipomatosis are usually asymptomatic and represent more of an aesthetic damage.
However, in some cases, they could cause pain and lead to unintended consequences. This occurs, for example, if lipomas exert pressure at the level of nerve endings, organs, tissues or blood vessels with their mass or with the possible increase in volume.
Precisely because of the possible compression exerted on organs and tissues, the liposome can give rise - depending on the area affected - to a series of serious complications that require timely medical intervention. These include:
- Difficulty swallowing;
- Respiratory problems;
- Cardiac disorders;
- Gastrointestinal disorders;
- Paralysis;
- Strong pain.
Finally, it should not be forgotten that - although rare - there is the possibility that lipomatosis may become complicated and evolve into malignant tumors.
Treatment
As mentioned, one of the possible causes of lipomatosis is to be found in hormonal and neurological imbalances. Consequently, if it were shown that the disorders affecting neurons and hormonal modulations were the real predisposing factors for lipomatosis, the pharmacological treatment of the disorder can proceed.
If lipomatosis cannot be resolved with drug therapy, then the surgical removal of fat accumulation and lipomas remains the only viable route.
To do this, cosmetic surgery techniques such as liposuction or liposculpture can be used.
Naturally, in the case in which the lipomatosis evolves into a malignant tumor form, the patient will have to undergo adequate anticancer therapies (pharmacological or not, in the opinion of the doctor).
