Introduction: Nosological Definition and Clinical Pictures of Lipedema
Nosological Definition and Clinical Pictures of Lipedema is typically defined as a volumetric increase localized mainly in the lower limbs, being bilateral, symmetrical, and of a soft consistency. It develops with the accumulation of adipose tissue in a pattern similar to “riding breeches,” extending from the hips to the ankles. Lipedema is a nosological entity of unknown etiology, predominantly affecting females. According to recent epidemiological studies, it manifests in about 11% of the female population, often beginning during menarche, menopause, or pregnancy.
Although less common in males, cases have been described with significant alterations in male sexual hormones and/or liver function disorders. Some authors highlight an increased incidence following cranial traumas and surgeries for pituitary adenomas.
Nosological Definition and Clinical Pictures of Lipedema can develop in an ascending or descending manner. The phenomenon begins at the hips and thighs, progressively worsening in the lower limbs. The typical sign of edemato-fibrosclerotic panniculitis, however, appears late. In advanced cases, particularly in untreated elderly subjects, subcutaneous nodular formations, a result of sclerotic processes affecting the subcutaneous connective tissue, can be observed. In severe cases, immobility is a direct consequence of elephantiasis in the lower limbs. Nosological Definition and Clinical Pictures of Lipedema The skin color is usually normal, except in cases where lipedema is associated with a particular form of erythrocyanosis (erythrocyanosis crurum puellarum). In some cases, hemispherical adipose cushions are evident medially at the knee joint. Occasionally, lipedema of the lower limbs is accompanied by lipedema of the upper limbs, typically developing from the shoulder and ending near the wrist. In rare cases, lipedema exclusively affects the upper limbs.
Other significant aspects defining lipedema include: Nosological Definition and Clinical Pictures of Lipedema
- Edematous fluid often accumulates in the affected limb, particularly during warm periods and in the latter half of the day. The lipedematous region is soft to manual pressure, causing pain in lipedema patients, unlike healthy individuals or those with lymphedema.
- Minor trauma, which normally leaves no trace in normal tissue, can cause ecchymosis in the lipedematous region, or hematomas localized in the subcutaneous adipose tissue.
- Lipedema often causes significant emotional stress: patients feel unattractive and deformed, negatively impacting their quality of life and leading to depression.
Physiopathological Aspects Nosological Definition and Clinical Pictures of Lipedema Adipose tissue is a particular type of connective tissue consisting of adipose cells (adipocytes), forming lobules separated by fibrous septa of connective tissue. Each adipocyte is supported by its own blood supply through a capillary system and innervated by adrenergic nerve fibers. It’s also believed that adipose tissue has sensory innervation. Lymphatic capillaries can be identified at the levels of the fibrous septa but not among the adipocytes. Few reticular fibers among the adipocytes constitute the initial (or prelymphatic) lymphatic drainage system.
Microangiopathy, which develops in the adipose tissue area causing increased protein permeability and capillary fragility, is one of the initial mechanisms distinguishing lipedema. As a consequence of increased permeability, high-protein fluid accumulates in the surrounding cellular area, while increased capillary fragility results in typical ecchymosis.
Nosological Definition and Clinical Pictures of Lipedema The soft consistency of adipose tissue may be associated with a neurogenic inflammatory state, further complicating the condition of microangiopathy. Some scholars believe this extremely soft consistency is linked to damage to the autonomic nervous system. This alteration could lead to erroneous interpretation of protopathic sensory inputs (related to pressure, temperature, or posture), as known, for example, in Sudeck-Leriche syndrome.
The pericellular fluid accumulation is associated with dilation of the prelymphatic drainage system, resulting in very slow lymphatic fluid drainage through the initial lymphatic vessels.
Typical pathological alterations in cutaneous lymphatic capillaries include very high capillary wall permeability. Studies using fluorescent microlinfangiography have shown aneurysm-like formations in association with numerous capillary segments of extremely reduced caliber.
Nosological Definition and Clinical Pictures of Lipedema: This explains how the dilation of prelymphatic channels, along with morphological and functional alterations of the lymphatic capillaries, suggests progressive damage to lymphatic drainage capacity.
Additionally, a disorder in the motor activity of the lymphangion (the anatomical-functional unit of the lymphatic collector, i.e., the segment between one valve apparatus and another) has been observed. Early stages, evidenced through oily contrast lymphangiographic examination, have shown the presence of wavy superficial lymphatic collectors, described as “corkscrew-shaped.” In cases associated with elephantiasis, the condition of progressive immobility exerts a detrimental effect on lymph formation and the motor activity of the lymphangion. Moreover, isotopic lymphography has shown an accelerated aging of the lymphatic pump.
In lipedema, skin elasticity is greatly reduced, while skin “compliance” (hardness index, measured in mmHg using a tonometer) is increased. This leads to severe consequences:
- The skin loses its role in aiding the venous pump of the lower limbs. In healthy subjects, in an upright position, pressure in the foot veins is about 100 mmHg; during walking, this pressure drops to about 30 mmHg. This mechanism does not function adequately in lipedema patients, and the resulting passive hyperemia leads to a high volume of ultrafiltration. Due to the increased skin “compliance,” a larger volume of interstitial fluid is needed to raise interstitial pressure, further compromising lymphatic drainage function and eliminating an important passive defense mechanism against edema development.
- Another significant aggravating factor is the absence of the veno-arteriolar reflex. Normally, in healthy subjects, this reflex causes vasoconstriction in orthostasis: the resulting reduction in the perfused capillary area, in turn, reduces the volume of ultrafiltrate in the lower limbs. Therefore, this absent reflex in lipedema represents a significant defense mechanism against the onset of edematous condition.
- Rarely are there macrophages in adipose tissue capable of eliminating plasma proteins outside the lymphatic vessels. As a result, fibrosis rapidly develops among the adipocytes, and collagen fibers appear.
- In lipedema, often, a pitting edema (fovea sign) develops in the second half of the day during warmer seasons. This is because heat causes the development of reactive hyperemia, which in turn increases the lymphatic water load. If edema associated with water retention joins lipedema, lymphatic vascular insufficiency becomes manifest. This is because good functionality of the valvular apparatus of the lymphatic vascular system promotes good lymphatic outflow and prevents gravitational reflux. Therefore, in lipedema, on the one hand, the lymphatic, water, and protein load is increased, lymph formation and lymphangion motor activity are altered, and on the other hand, the valvular apparatus of the lymphatic system is severely insufficient.
Complications and Association with Other Pathologies Complications related to the patient:
- Anorexia nervosa
Complications related to doctors: Nosological Definition and Clinical Pictures of Lipedema
- Sclerotherapy (telangiectasia)
- Varicectomies without absolute indication
- Gastric banding, gastric bypass
Natural complicationsNosological Definition and Clinical Pictures of Lipedema : Nosological Definition and Clinical Pictures of Lipedema
- Lipedema + idiopathic edema syndrome
- Lipedema + osteoarthritis
- Lipedema + chronic venous insufficiency
FIG.1, A-F: CLINICAL, RADIOLOGICAL Nosological Definition and Clinical Pictures of Lipedema (LYMPHANGIO-CT, RETROGRADE DUCTOGRAPHY), SURGICAL (NEEDLE CANNULA INSERTED INTO THE ORIFICE CORRESPONDING TO THE CHYLOUS FISTULA AND CHYLOCELE CAPSULE), AND LONG-TERM RESULTS RELATING TO A TRAUMATIC LESION OF THE THORACIC DUCT.
Therapy It’s important to emphasize that lipedema is not merely a “subjective problem” of the patient, nor just a matter of excessively adipose lower limbs, nor a simple constitutional variation, as sometimes claimed, but a pathology that requires appropriate treatment to avoid possible complications.
Nosological Definition and Clinical Pictures of Lipedema Complete decongestive therapy (CDT), characterized by manual lymphatic drainage, the use of suitable graduated compression supports and/or functional bandages, therapeutic exercises, and meticulous skin hygiene, is initially contraindicated in cases where obesity, generally associated with significant anatomical-functional alterations of the cardiovascular, pulmonary, and musculoskeletal systems, is present as a comorbidity. This includes, in particular, arterial hypertension, congestive heart failure, diabetes mellitus, hyperlipidemia, Pickwick syndrome, osteoarthritis of the hips, knees, or feet. In these cases, where a combined form of lipedema and obesity exists, it is advisable to begin complete decongestive therapy after carefully correcting the disorders related to obesity, not only with medical-conservative aids, through a suitable body weight reduction program, but also, and above all, surgically. Obesity treatment is, in fact, essential so that complex decongestive therapy, associated with microsurgical treatment, is successful.
Nosological Definition and Clinical Pictures of Lipedema Nosological Definition and Clinical Pictures of Lipedema, The surgical treatment of obesity experienced a groundbreaking shift with the conception and introduction of biliopancreatic diversion (Biliopancreatic Diversion, BPD) by Prof. Nicola Scopinaro and his collaborators at the Surgical Clinic of the University of Genoa. This marked a fundamental qualitative shift in the surgical treatment of obesity, as it guarantees a true resolution of the pathological condition through a functional regulation of energy intake and absorption, resulting in significant weight loss associated with a permanent normalization of blood glucose and cholesterol levels in all cases.
Conclusion and Key Points: Nosological Definition and Clinical Pictures of Lipedema
- Predominantly Affects Women: Often emerging during hormonal changes such as menarche, pregnancy, or menopause.
- Characterized by Symmetrical Accumulation of Adipose Tissue: Typically affects the lower limbs, presenting as a ‘riding breeches’ appearance.
- Physiopathology: Involves microangiopathy and potentially a neurogenic inflammatory state, along with alterations in lymphatic capillaries and the prelymphatic drainage system.
- Complications and Associations: Can be associated with conditions like anorexia, bulimia, and various vascular disorders. Mismanagement or inappropriate treatment approaches (like liposuction without indication) can lead to further complications.
- Therapy: A holistic approach is essential, involving decongestive therapies and, in cases of concurrent obesity, weight management strategies including surgical interventions like biliopancreatic diversion.
Effective management of Nosological Definition and Clinical Pictures of Lipedema , addressing both the physical symptoms and the psychological impact of the condition. Early recognition and proper treatment are key to improving quality of life and preventing complications.
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