Lipedema | Definition | Therapy
NOSOLOGICAL DEFINITION AND CLINICAL PICTURES
Lipoedema can be defined as a volumetric increase, typically localized in the lower limbs, bilateral, symmetrical, with a soft consistency, which develops with the accumulation of adipose tissue in a similar way to "breeches", ie starting from the hips up to at the level of the ankles. Lipoedema is a nosological entity of unknown etiology that predominantly affects the female sex. According to recent epidemiological studies, it occurs in 11% of the female population: it frequently begins at the time of menarche, menopause or during pregnancy.
Although a low incidence has been found in males, cases characterized by a significant alteration of male sex hormones and / or disturbances in liver function have been reported. In particular, some authors underline the existence of an increased incidence following head trauma and subsequent surgery for pituitary adenomas.
Lipoedema can have an ascending or descending development. The phenomenon begins at the level of the hips and thighs, with worsening progression in the lower limbs. The typical sign constituted by edemato-fibrosclerotic panniculitis appears, however, late. In advanced cases, especially in untreated elderly subjects, subcutaneous nodular formations may be appreciable, the result of sclerotic processes affecting the subcutaneous connective tissue. In severe cases, immobility is the direct consequence of elephantiasis affecting the lower limbs. Skin color is usually normal, except in cases where lipoedema is associated with a particular form of erythrocyanosis (erythrocyanosis crurum puellarum). In some cases, hemispheric fat pads are evident medially at the knee joint. Occasionally, lipedema of the lower limbs is accompanied by lipedema of the upper limbs and, typically, swelling develops from the shoulder ending near the wrist. In rare cases, however, lipedema affects only the upper limbs.
We can also distinguish other significant aspects that define lipedema.
Often the edematous fluid accumulates in the affected limb and this, in particular, in hot periods and in the second half of the day. In the lipedematous region a soft consistency is appreciable under manual pressure. This pressure does not cause a painful sensation to the healthy subject or to the patient with lymphedema , while it arouses a painful sensation in the patient with lipedema.
A minor trauma that normally leaves no trace in normal tissue can, on the other hand, cause bruising in the lipedematous region, or hematomas localized in the subcutaneous adipose tissue.
Frequently lipedema causes a strong state of emotional stress: the patient feels unpleasant and deformed, a condition that negatively affects the quality of life and the depression that arises, consequently, in these subjects leads to further complications.
PHYSIOPATHOLOGICAL ASPECTS
Adipose tissue is a particular type of connective tissue made up of adipose cells (adipocytes) which, grouping together, form lobules separated by fibrous septae of connective tissue. Each single fat cell has its own blood support guaranteed by a capillary system and its own innervation mediated by adrenergic nerve fibers. It is also argued that adipose tissue also has sensory innervation. Lymphatic capillaries can be seen at levels of the fibrous septa, but not among the adipocytes. The few reticular fibers between the fat cells constitute the initial (or prelymphatic) lymphatic drainage system.
Microangiopathy, which develops in the area of adipose tissue causing an increase in protein permeability and, likewise, an increase in capillary fragility, constitutes one of the initial mechanisms that distinguish lipedema. As a consequence of the increase in permeability, the accumulation of liquid with a high protein content in the surrounding cellular area occurs, while as a consequence of the increase in capillary fragility, the typical bruises appear.
The soft consistency of the adipose tissue can be associated with a neurogenic inflammatory state, which further complicates the condition of microangiopathy. Some scholars are of the opinion that this extremely soft texture is linked to damage to the autonomic nervous system. According to this theory, this alteration could lead to an erroneous interpretation of the protopathic sensory inputs (related to pressure, temperature or posture) and this is known, for example, in Sudeck-Leriche syndrome.
The pericellular accumulation of fluid is associated with dilation of the pre-lymphatic drainage system, with the result that the fluid flows only very slowly through the initial lymphatic vessels.
Typical pathological alterations are appreciable at the level of the cutaneous lymphatic capillaries. In particular, the permeability of the capillary wall is very high and some studies, characterized by the use of fluorescent microlymphangiography, have highlighted aneurysm-like formations in association with the evidence of numerous capillary segments consisting of extremely small caliber.
In this way it can be understood how the dilation of the prelymphatic channels, together with the morphological and functional alterations of the lymphatic capillaries, suggest a progressive damage to the lymphatic drainage capacity.
In addition to this, a disturbance of the motor activity of the lymphangion was observed (which represents the anatomo-functional unit of the lymphatic collector, or the segment interposed between one valve apparatus and the other). Early pictures, highlighted by the use of lymphangiographic examination with oily contrast, showed the presence of suprafascial lymphatic collectors with a wavy shape, so-called "corkscrew". In cases associated with elephantiasis, the condition of progressive immobility exerts a detrimental effect on the formation of lymph on the motor activity of the lymphangion. Furthermore, isotopic lymphography showed accelerated aging of the lymphatic pump.
In lipedema the skin elasticity is strongly reduced, while the skin “compliance” (hardness index, expressed in mmHg, measured by tonometer) is increased. This has serious consequences:
The skin loses its helping role as a venous pump of the lower limbs. In healthy subjects, in an upright position, the pressure in the veins of the back of the foot is equal to 100 mmHg; during walking this pressure drops to a value of 30 mmHg. This mechanism does not function adequately in patients with lipedema and the resulting passive hyperemia leads to a high volume of ultrafiltration. Due to increased skin compliance, a larger volume of interstitial fluid is required to raise interstitial pressure. In this way the lymph drainage function is further compromised and, consequently, an important passive defense mechanism against the development of edema is lost.
Another significant aggravating factor is the fact that the veno-arteriolar reflex is absent. Normally, in healthy subjects, this reflex involves vasoconstriction in orthostasis: the resulting reduction in the perfused capillary area in turn reduces the volume of ultrafiltrate in the lower limbs. Therefore this reflex, lacking in lipedema, represents an important defense mechanism against the onset of the edematous condition.
In adipose tissue there are rarely macrophages capable of eliminating plasma proteins outside the lymphatic vessels. For this reason, fibrosis develops rapidly between the adipocytes and collagen fibers appear.
In lipedema, an imprint edema (sign of the fovea) often develops in the second half of the day during the more temperate seasons. The reason for this is linked to the fact that heat leads to the development of reactive hyperemia which, in turn, increases the lymphatic water load. If an edema associated with water retention is associated with a lipedema, the lymphatic vascular insufficiency becomes manifest. This is because a good functionality of the valvular apparatus of the lymphatic vascular system favors a good lymphatic outflow and prevents gravitational reflux. Therefore, in lipedema, on the one hand the lymphatic, water and protein load is increased, the formation of lymph and the motor activity of the lymphangion are altered, and on the other hand the valvular apparatus of the lymphatic system is severely insufficient.
COMPLICATIONS AND ASSOCIATION WITH OTHER PATHOLOGIES
Patient-related complications:
Nervous anorexia
Bulimia
Complications related to doctors:
Diuretics / laxatives
Lipectomy
Liposuction
Sclerotherapy (telangiectasias)
Varicectomies without absolute indication
Gastric band, gastric bypass
Natural complications:
Lipo- lymphedema
Lipedema + idiopathic cidic edema syndrome
Lipedema + arthrosis
Lipedema + chronic venous insufficiency
THERAPY
It should be emphasized that lipedema is not exclusively a "subjective problem" of the patient, nor a single issue of excess lower limbs in fat, nor a simple constitutional variation, as is sometimes claimed, but it represents a pathology that requires appropriate treatment in order to avoid possible complications.
Complete decongestive therapy (CDT), characterized by manual lymphatic drainage, by the use of adequate gradual compression elastic braces and / or valid functional bandages, by therapeutic exercises and by meticulous skin hygiene, is initially contraindicated in cases where it is present, such as comorbidity, the condition of obesity, generally associated with important anatomo-functional alterations in the cardiovascular, pulmonary and musculoskeletal systems including, in particular, arterial hypertension, congestive heart failure, diabetes mellitus, hyperlipidemia, Pickwick's syndrome, osteoarthritis of the hips, knees or feet. In these cases where, therefore, there is a combined form of lipedema and obesity, it is advisable to start the complete decongestant therapy after having carefully corrected the disorders related to obesity, not only with medical-conservative measures, through an appropriate program of reduction of body weight, but also and above all of a surgical nature. The treatment of obesity is, in fact, essential for complex decongestive therapy, associated with microsurgical treatment, to be successful.
In selected cases it is possible to give an appropriate indication to bariatric surgical treatment with global improvement of lipedema.