Your search

A grossly obese woman was wrongly diagnosed throughout her adult life of having lymphoedema. Her condition was subsequently confirmed as lipoedema, an entirely different condition, which is noted in medical text books but is seldom taught to medical students or to general practitioners. The condition is caused by abnormal deposition of adipose tissue in the extremities (usually the lower limbs) and almost exclusively affects women. It often starts at puberty or may occur after pregnancy. The exact aetiology is not yet understood but genetic and hormonal factors may be implicated. The problem is that misdiagnosis leads to inappropriate tests and improper treatment to the patient. When recognised it is often too late to do anything for the patient and they become highly dependent on social care. This case describes how the diagnosis can be confirmed through an ultrasound image and illustrates the need for early recognition to facilitate specialist care.

Lipoedema UK was founded in 2012 by women with lipoedema and clinicians working in the Lymphoedema Service at St George's Hospital in London. Its patron is Professor Peter Mortimer, the UK's leading Lipoedema expert, and its nurse consultant is Sandy Ellis, who diagnoses and treats many women with Lipoedema in the UK and is also the nurse consultant in the St George's team. The charity's objectives are to educate doctors, health professionals and the public about lipoedema and its symptoms, so it may be diagnosed and treated earlier.

Due to its increased presence in the press and on television, the diagnosis of lipedema is on the way to becoming a trendy diagnosis for those with thick legs. Despite this, one must recognize that lipedema is a very rare disease. It is characterized by disproportional obesity of the extremities, especially in the region of the hip and the legs, hematoma development after minimal trauma, and increased pressure-induced or spontaneous pain. Aids for making the correct diagnosis are (duplex) sonography, the waist-hip index or the waist-height index and lymphoscintigraphy. Important differential diagnoses are constitutional variability of the legs, lipohypertrophy in obesity, edema in immobility, edema in chronic venous insufficiency and rheumatic diseases. The symptom-based therapy of lipedema consists of conservative (compression, manual lymphatic drainage, exercise) and surgical treatments (liposuction). Until now there is no curative therapy. Obesity is an important risk factor for the severity and prognosis of lipedema. Further studies for a better understanding of the pathogenesis of lipedema and in the end possible curative treatments are urgently needed.

In the differential diagnosis of lymphedema, lipedema is often mentioned; however, in lipedema, there is initially no primary lymphatic impairment due to dysfunction. In the later stages of lipedema, obesity is often involved and will influence the patient negatively. In daily practice, contrary to the treatment point of view, a therapeutic approach of manual lymph drainage and compression therapy is often used for both lipedema and lymphedema, although these are two distinguishable diagnoses. Therefore, differentiating these two conditions is crucial for an optimal, dedicated treatment program. Because there is no consensus on the criteria for the diagnosis lipedema, a new method should be used to categorize and stratify patients to offer a dedicated treatment program and psycho-social support. The WHO method of International Classification of functioning, disability and health (ICF) is designed for a new approach of chronic diseases and can be of help in patients with lipedema. Lipedema is a debilitating, incurable chronic pathological condition that is often misdiagnosed or unrecognized. The most common form of symmetrical fat distribution is obesity. However, many diseases and syndromes, such as Cushing’s disease21 and polycystic ovary syndrome22, can lead to unusual fat deposition patterns. Lipedema almost exclusively affects women and is characterized by a bilateral, progressive accumulation and misdistribution of subcutaneous fat, usually below the waist. Only two cases of lipedema in male patients have been reported. The onset of lipedema is often soon after puberty; however, lipedema can develop later in life, such as during pregnancy or menopause. During the course of lip edema, very often a obesity component will be present. As a clinical syndrome, lipedema was first described as the presence of excessive fat deposits on the buttocks, thighs and legs associated with mild edema. Once accumulated, the excessive fat deposits respond poorly to vigorous dietary measures. Dietary measures predominately reduce fat in the areas of the body without lipedema. This process leads to an asymmetrical distribution of body fat between the upper body and lower extremities, a disproportional fat distribution. Because of the diet-resistant nature of lipedema part, many patients are reluctant to pursue stringent dietary measures, as this emphasizes the disproportionate body fat. Approximately 50% of lipedema patients have an elevated body mass index (BMI), which complicates the differentiation between lipedema and obesity (from the Latin obesus, grown fat by eating). Nevertheless, very often volume reduction of a lipedema leg is achieved after a conservative treatment program and weight reduction. In addition, large accumulations of subcutaneous fat deposits can mechanically compress existing and previously functional lymphatic structures, leading to a mechanical insufficiency and secondary lymphedema over time. In practice, diagnosing lipedema is often complicated, but lipedema must be differentiated from lipohypertrophy, lymphedema and obesity to provide the patient with adequate treatment options. The exact etiology of lipedema is still unknown, although recent research has suggested the involvement of several genetic factors. Because lipedema is often not recognized or misdiagnosed, the scarcely available prevalence figures are likely a significant underestimation. All of the current treatment options are non-curative, complex and require a multidisciplinary approach. Surgical treatment of the affected limbs occurs when conservative treatment options are no longer effective and daily functioning becomes severely compromised.

BACKGROUND: The management of lymphoedema is complex and should be based on guidelines. To date, no data assessing quality of care in lymphoedema in Germany are available. OBJECTIVE: We aimed at evaluating the quality of care of lymphoedema in the metropolitan area of Hamburg using guideline-based indicators. METHODS: Cross-sectional, community-based study including patients with lymphoedema. Assessment included a structured interview, clinical examination and patient-reported outcomes. Quality indicators derived from guidelines by a Delphi consensus were applied. RESULTS: 348 patients (median age 60.5 years) with lymphoedema (66.4%), lipoedema (9.5%) or combined oedema (24.1%) were included. 86.4% performed compression therapy, 85.6% received lymphatic drainage. On average 55.0% of the quality of care criteria were met; 64.8% were satisfied with care. The distribution curve of the health care index was almost normal. Treatment by specialists led to a higher quality of care index. CONCLUSION: Although overall quality of care in lymphoedema is fair, many patients are not treated properly according to guidelines.

Classification, lipoedema, obesity, oedema.

Background: Butcher’s broom plant extract has been reported to improve lymphatic flow and the trace mineral, selenium, has been shown to improve lymphedema. This retrospective case study examines the effectiveness of Butcher’s broom in conjunction with selenium to decrease limb volume of a patient with lipedema, a common fat distribution disorder with excess adipose tissue fluid. Methods: Selenium (400 mcg) was initiated 6 days prior to limb volume evaluation utilizing perometry. The patient underwent physical therapy that consisted of manual lymph drainage (MLD) with Histological Variable Manual Technique (HIVAMAT), and compression bandaging. Butcher’s broom (one gram daily) was added on day 95 of treatment in addition to selenium and both were continued through day 293 (end of study). Results: Total volume reduction over the study period for the left and right upper extremities and left and right lower extremities was 525 ml and 225 ml (p<0.05), and 1769 ml and 1614 ml (p<0.0001), respectively. The total percent volume reduction during the time period when MLD with HIVAMAT and compression bandaging were performed for the left and right legs was 70.6 and 79.0%, respectively. In the absence of compression bandaging, the left and right arms lost 21.2 and 10% of initial volumes, respectively at the 6 month follow-up visit. During the latter part of the study when the patient was performing a home maintenance program, at which time selenium and Butcher’s broom were continued, the left and right lower extremities decreased an additional 29.4 and 20.9% of initial volumes, respectively, despite a lack of exercise due to a foot injury during the last 46 days. Conclusion: Butcher’s broom and selenium may offer new tools in conjunction with physical therapy to improve swelling and pain associated with lipedema.

Background: Butcher’s broom plant extract has been reported to improve lymphatic flow and the trace mineral, selenium, has been shown to improve lymphedema. This retrospective case study examines the effectiveness of Butcher’s broom in conjunction with selenium to decrease limb volume of a patient with lipedema, a common fat distribution disorder with excess adipose tissue fluid. Methods: Selenium (400 mcg) was initiated 6 days prior to limb volume evaluation utilizing perometry. The patient underwent physical therapy that consisted of manual lymph drainage (MLD) with Histological Variable Manual Technique (HIVAMAT), and compression bandaging. Butcher’s broom (one gram daily) was added on day 95 of treatment in addition to selenium and both were continued through day 293 (end of study). Results: Total volume reduction over the study period for the left and right upper extremities and left and right lower extremities was 525 ml and 225 ml (p<0.05), and 1769 ml and 1614 ml (p<0.0001), respectively. The total percent volume reduction during the time period when MLD with HIVAMAT and compression bandaging were performed for the left and right legs was 70.6 and 79.0%, respectively. In the absence of compression bandaging, the left and right arms lost 21.2 and 10% of initial volumes, respectively at the 6 month follow-up visit. During the latter part of the study when the patient was performing a home maintenance program, at which time selenium and Butcher’s broom were continued, the left and right lower extremities decreased an additional 29.4 and 20.9% of initial volumes, respectively, despite a lack of exercise due to a foot injury during the last 46 days. Conclusion: Butcher’s broom and selenium may offer new tools in conjunction with physical therapy to improve swelling and pain associated with lipedema.

SUMMARY: 1. The muscular atrophy leads across the Fascia brachialis and retinacula cutis to substantial sagging of the upper arm. 2. The posterior deep fat compartment sags together with the Fascia brachialis over the triceps muscle. 3. The lipedema manifestation is often ignored when planning surgery of the upper arm. 4. The anatomical structures are important therapeutic options.

Edema is an accumulation of fluid in the interstitial space that occurs as the capillary filtration exceeds the limits of lymphatic drainage, producing noticeable clinical signs and symptoms. The rapid development of generalized pitting edema associated with systemic disease requires timely diagnosis and management. The chronic accumulation of edema in one or both lower extremities often indicates venous insufficiency, especially in the presence of dependent edema and hemosiderin deposition. Skin care is crucial in preventing skin breakdown and venous ulcers. Eczematous (stasis) dermatitis can be managed with emollients and topical steroid creams. Patients who have had deep venous thrombosis should wear compression stockings to prevent postthrombotic syndrome. If clinical suspicion for deep venous thrombosis remains high after negative results are noted on duplex ultrasonography, further investigation may include magnetic resonance venography to rule out pelvic or thigh proximal venous thrombosis or compression. Obstructive sleep apnea may cause bilateral leg edema even in the absence of pulmonary hypertension. Brawny, nonpitting skin with edema characterizes lymphedema, which can present in one or both lower extremities. Possible secondary causes of lymphedema include tumor, trauma, previous pelvic surgery, inguinal lymphadenectomy, and previous radiation therapy. Use of pneumatic compression devices or compression stockings may be helpful in these cases. (Am Fam Physician. 2013;88(2):102-110. Copyright © 2013 American Academy of Family Physicians.)

A 54-year-old female patient reported that a characteristic of her family was 'fat legs' with postural edema since adolescence. Over the years the patient had been gaining weight with an increase in fatty tissue in the legs and arms. At the age of 24 years she started taking oral contraceptives and noted worse swelling and pain in the lower limbs. She was advised to suspend the use of the contraceptives and to start using a transdermal lymphatic system drug and physical exercise which partially improved the symptoms. Three years ago she noted that the swelling was increasing without improvement and sought a physician who raised the hypothesis of lymphedema and referred her to a specialized center. Lipedema and lymphedema was diagnosed in the physical examination. A 3-day intensive treatment program (8 h daily) was started for lymphedema which included manual and mechanical lymph drainage associated with short-strech (<50 mm Hg) compression stockings custom made using a cotton-polyester fabric. Volumetry and perimetry were performed before starting and after the treatment and the legs were photographed. Volumetric and perimetric reductions were obtained suggesting the involvement of regional cutaneous lymphostasis in this disease.

Edema of the limbs is a common reason for medical consultation, for which the lymphoscintigraphy is considered to be a reliable method for its differential diagnosis. OBJECTIVE: To evaluate the usefulness of radionuclide studies in the differential diagnosis of edema, and the diagnostic yield of different scintigraphic patterns. MATERIAL AND METHODS: A total of 61 patients, mean age 43 years, referred to our Department in the last three years for suspected lymphoedema, were considered. One patient was discarded due to lack of diagnosis, 56 had lower limb edema and 4 upper limb edema. After intradermal injection of two doses of (99m)Tc-nanocolloid, scintigraphic scans were made at 30 and 120minutes. The final diagnosis was based on imaging tests, clinical course, and response to treatment. We calculated the parameters of the diagnostic yield of four different scintigraphic patterns (presence of dermal backflow, asymmetry-alteration in inguinal/axillary nodes, presence of collateral pathways, and visualization of intermediate lymph nodes), considering them individually and jointly. RESULTS: The best diagnostic yield was achieved by considering dermal backflow and asymmetry in inguinal/axillary nodes (accuracy 88.9%, specificity 96.4%, PPV 95.5%). Evaluation of intermediate lymph nodes and presence of collateral pathways contributed little to the diagnostic yield, showing poor sensitivity and high false positive rates. CONCLUSION: The lymphoscintigraphy had high diagnostic yield, allowing early treatment of lymphœdema. The dermal backflow and asymmetry in inguinal/axillary nodes had the greatest diagnostic accuracy. Evaluation of intermediate lymph nodes and visualization of collateral pathways contributed little to improving the diagnosis.

The pathophysiology of skin diseases associated with monoclonal gammopathies is generally unknown. Our aim was to investigate whether a monoclonal gammopathy could be a causal factor in progressive lymphedema. We describe a 75 year old patient with a rapidly progressive lipo-lymphedema and a monoclonal gammopathy of unknown significance (MGUS) suspected as a key etiological factor. Dermal fibroblasts were cultured from lesional lower leg skin and non-lesional abdominal skin and compared to healthy control fibroblasts. We found 10-fold elevated basic fibroblast growth factor 2 (FGF-2) in the patient's serum and significantly increased basal FGF-2 production of lesional and non-lesional fibroblasts compared to healthy controls. Upon restimulation with patient or healthy control serum, lesional fibroblasts showed significantly increased proliferation rates and FGF-2 production in vitro. Non-lesional abdominal fibroblasts showed an intermediate phenotype between lesional and control fibroblasts. Our findings provide the first evidence that lesional dermal fibroblasts from lipo-lymphedema with plasma cell infiltration show increased proliferation and FGF-2 production and that both local tissue factors and altered FGF-2 serum levels associated with monoclonal gammopathies might contribute to this phenotype. Thus we propose a possible pathophysiologic link between the gammopathy-associated factors and the generation of lymphedema with initial fibrogenesis aggravating pre-existing lipedema.

Lipedema is a clinical entity frequently misdiagnosed or confound with primary lymphedema. Lipedema is a disorder of adipose tissue that occurs almost exclusively in obese women. It is characterized by bilateral enlargement from hip to ankle due to abnormal depositions of subcutaneous fat associated with often mild edema, usually sparing the feet. Disease onset is usually at or soon after puberty. Lipedema results in considerable frustration and distress resulting from the cosmetic appearance. Patients may complain of pain, tenderness, easy bruising of the affected areas with moderate to severe sensitivity to digital pressure or pinching. Imaging studies using computed tomography, magnetic resonance imaging, ultrasound, lymphoscintigraphy are not indicated, except if the diagnosis is atypic or doubtful. Long-term evolution may alter lymphatic system and lead to a lipo-lymphedema with specific complications such as cellulitis. Lipedema management is not codified and included weight loss (poorly improving leg appearance or discomfort), psychological counselling and compression therapy. Liposuction, especially using tumescent local anaesthesia, may reduce edema, spontaneous pain, sensitivity to pressure, bruising and improve appearance resulting in a important increase in quality of life.

Lipedema is a disproportionate, symmetrical fatty swelling characterized by pain and bruising existing almost exclusively among women. We undertook a systematic review of the available literature about lipedema, given the lack of knowledge and little evidence about this disorder especially among obesity experts. Diagnosis of lipedema is usually based on clinical features. Symmetrical edema in the lower limbs with fatty deposits located to hips and thighs usually appears at puberty and often affects several members of the same family. Main disorders considered for differential diagnosis are lymphedema, obesity, lipohypertrophy and phlebedema. Treatment protocols comprise conservative (decongestive lymphatic therapy) and surgical (liposuction) approaches. Early diagnosis and treatment are mandatory for this disorder otherwise gradual enlargement of fatty deposition causes impaired mobility and further comorbidities like arthrosis and lymphatic insufficiency.