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Is Lipedema a Unique Entity?

Authors:
  • Amato - Instituto de Medicina Avançada

Abstract

Lipedema is a disease with high prevalence but low recognition. It is often misdiagnosed and underdiagnosed. Obesity and lymphoedema are the most common differential diagnoses and can also coexist in patient with lipedema. Its broad range of presentation and fat distribution types contribute to this confusion. It is likely that lipedema symptom variations and presentation forms are often associated with hormonal variations, chronic low-grade systemic inflammation, and wide polygenic variations. This paper presents a theory regarding the clinical evolution of lipedema clinical and its involvement with other diseases, suggesting a three-phase approach for treatment.
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... Lipedema, primarily characterized by an abnormal accumulation of fat in the legs, often leads to discomfort and a feeling of swelling, particularly when standing [1]. The root cause of lipedema is yet to be clearly understood, but it is known to be linked with inflammation [2,3]. Frequently, lipedema is misdiagnosed as obesity, gynoid lipodystrophy, or lymphedema, leading to its inadvertent oversight in the preliminary stages of medical evaluations. ...
... It is well known that initial lipedema onset can be triggered by hormonal changes such as puberty, pregnancy, and menopause, although the exact cause remains unknown [14]. Additionally, inflammatory symptoms associated with lipedema can be both triggered and alleviated by various factors [2], including dietary approach, where many of them recommend reducing the consumption of carbohydrates, which are rich in gluten. Our study suggests a possible association between the presence of either HLA-DQ2 or HLA-DQ8 -or both -and the inflammation seen in lipedema, especially when combined with gluten intake. ...
... Rather than relying on inflammatory lab tests, which can be effectively obscured by lipedema, we chose a different path by focusing on symptomatic, inflamed lipedema. It is important to point out that our study indicates a higher incidence of CD-associated HLA alleles in lipedema patients seeking medical assistance, which corroborates the idea of various inflammatory triggers [2]. Nonetheless, this finding does not establish a causal relationship; CD-associated HLA alleles (HLA-DQ2 and HLA-DQ8) do not appear to be included in the genes necessary for diagnosing lipedema, as 39% of lipedema patients had no such HLA alleles present. ...
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Objective The aim of this study is to assess the prevalence of HLA-DQ2 and HLA-DQ8 in women diagnosed with lipedema. Methods Leukocyte histocompatibility antigen (HLA) tests of 95 women diagnosed with lipedema were analyzed using non-probabilistic sampling for convenience. The prevalence of HLA-DQ2 and HLA-DQ8 was compared to the general population. Results The prevalence of HLA-DQ2+ was 47.4%, that of HLA-DQ8+ was 22.2%, the presence of any celiac disease associated HLA (HLA-DQ2+ or HLA-DQ8+) was 61.1%, both HLA (HLA-DQ2+ and HLA-DQ8+) was 7.4%, and the absence of celiac disease associated HLA was 39%. Compared to the general population, there was a significantly higher prevalence of HLA-DQ2, HLA-DQ8, any HLA, and both HLAs in lipedema patients. The mean weight of patients with HLA-DQ2+ was significantly lower than the overall study population, and their mean BMI significantly differed from the overall mean BMI. Conclusion Lipedema patients seeking medical assistance have a higher prevalence of HLA-DQ2 and HLA-DQ8. Considering the role of gluten in inflammation, further research is needed to establish if this association supports the benefit of gluten withdrawal from the diet in managing lipedema symptoms.
... The current study examined the overlap between lipedema symptoms and attention-deficit/hyperactivity disorder (ADHD). Lipedema is characterized by the abnormal accumulation of fat in the lower limbs, which may be accompanied by complaints of pain and edema when standing up [1,2]. The cause of lipedema is not well understood, but it is known to be linked to inflammation. ...
... The current self-report measures for assessing the presence of ADHD and lipedema have limitations. Lipedema inflammation is cyclical [2], so it may influence ADHD more at certain times. The worst time to decide on surgical treatment may be during times of peak inflammation, as inflammation could significantly impact cognitive function at these times. ...
... That way, we can better understand lipedema's neurophysiological mechanisms. This includes exploring inflammation's role in ADHD and lipedema and developing methods for better measuring low-grade chronic inflammation [2]. Incorporating mental health considerations into treatment approaches for lipedema may be beneficial [22], and routine screening for ADHD in lipedema patients may improve clinical treatment strategies. ...
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Introduction The current study aimed to investigate the overlap between symptoms of lipedema and attention-deficit/hyperactivity disorder (ADHD). Lipedema is a condition that causes abnormal fat accumulation and inflammation in the legs and buttocks, often accompanied by edema and pain. ADHD is a common condition characterized by difficulty paying attention and controlling behavior, affecting the social, academic, and occupational quality of life. The study’s primary objective was to assess the prevalence of ADHD symptoms in a population of women with lipedema symptoms and compare the clinical characteristics. Method The study used a lipedema screening questionnaire and the Adult Self-Report Scale (ASRS-18) to assess the prevalence of ADHD in a sample of 354 female volunteers with or without a prior lipedema diagnosis. Results Of the lipedema group, 100 (77%) were ASRS positive, and 30 (23%) were ASRS negative. In the group without lipedema, 121 (54%) were ASRS positive, and 103 (46%) were ASRS negative, with a relative risk of 1.424 (p<0.0001). Conclusion Our results demonstrate a positive correlation between lipedema and ADHD and suggest that targeted strategies to improve clinic attendance for individuals with ADHD may improve lipedema treatment outcomes. Patients with lipedema symptoms are more likely to have ADHD symptoms.
... Lipedema was described in Brazil by Moraes as lipophilia membralis, preferential fat deposition in lower limbs. 1 It is characterized by the deposition of abnormal fat in the lower and upper limbs bilaterally, and may be accompanied by complaints of orthostatic edema in women after puberty. 2 The pathophysiology of lipedema is poorly understood; nevertheless it is described as lymphatic compromise in its initial stages, 3 and as frank lymphatic damage in the final stage of lipolympedema. 4 It is a disease with a high prevalence in the population, estimated at 11% of the female population by F€ oldi et al. 5 Lipedema has genetic characteristics 6 and a clear hormonal influence resulting in chronic low-grade inflammatory symptoms. 2 Despite its distinct nature, it is often confused with more commonly diagnosed diseases such as obesity, venous insufficiency, and lymphedema, 7,8 all of which have distinct clinical characteristics. ...
... Lipedema was described in Brazil by Moraes as lipophilia membralis, preferential fat deposition in lower limbs. 1 It is characterized by the deposition of abnormal fat in the lower and upper limbs bilaterally, and may be accompanied by complaints of orthostatic edema in women after puberty. 2 The pathophysiology of lipedema is poorly understood; nevertheless it is described as lymphatic compromise in its initial stages, 3 and as frank lymphatic damage in the final stage of lipolympedema. 4 It is a disease with a high prevalence in the population, estimated at 11% of the female population by F€ oldi et al. 5 Lipedema has genetic characteristics 6 and a clear hormonal influence resulting in chronic low-grade inflammatory symptoms. 2 Despite its distinct nature, it is often confused with more commonly diagnosed diseases such as obesity, venous insufficiency, and lymphedema, 7,8 all of which have distinct clinical characteristics. ...
... The diagnosis of lipedema remains primarily clinical, using the standardization of pertinent clinical questions from the QuASiL questionnaire 13 associated with the following criteria for classification of the group with the disease: suggestive clinical history in women after puberty; with bilateral symmetrical fat deposit below the hip, sparing of the feet (negative Stemmer sign); non-depressible edema (negative Godet's sign), resistance to elevation of the limbs; painful affected areas that are sensitive to palpation; and increased capillary fragility, with spontaneous bruising. 2,4 Imaging protocol ...
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Background Lipedema is characterized by the deposition of abnormal fat in the lower and upper limbs bilaterally. It is a disease with high prevalence and genetic characteristics. Non-specific and non-quantified increases in the thickness of the subcutaneous tissue have previously been demonstrated using magnetic resonance imaging and computed tomography. Objectives To evaluate the thickness of the dermis and subcutaneous tissue in predetermined areas as a distinguishing feature between individuals with and without lipedema using ultrasound. Methods Ultrasound images of 89 female patients were analyzed, including patients undergoing clinical investigation for venous insufficiency or lipedema who underwent ultrasound evaluations at our institution. Patients were divided in two groups: with lipedema clinically diagnosed and those without lipedema. They underwent a common Doppler protocol for venous mapping to assess venous insufficiency associated with the evaluation of dermis and subcutaneous thickness at pre-defined points of the lower limbs. Results There were 63 patients with lipedema. Anterior thigh, pre-tibial and lateral aspect of the leg and supra-just medial malleolar region were significantly different. Supra-just medial malleolar region was significantly different with BMI above 25. An optimal cutoff value was calculated for the ultrasound diagnosis of lipedema using thickness of the dermis and subcutaneous tissues. Conclusions Studied criteria allow use of simple and reproducible ultrasound cutoff values to diagnose lipedema in the lower limbs. Pre-tibial region thickness measurement, followed by thigh and lateral leg thickness are recommended for the ultrasound diagnosis of lipedema.
... 1,2 The pathophysiology and epidemiology of lipedema remain little understood. 4,5 Moreover, lipedema was only recently included in the 11th revision of the International Classification of Diseases (ICD-11) (EF02.2 and BD93.1Y) 6 and therefore is not yet part of the academic curriculum of medical degrees in Brazil, nor of the vascular specialty curriculum. It is thus still often confused with other more common conditions, such as obesity, gynoid lipodystrophy, and lymphedema, 7,8 and is rarely diagnosed at the first medical consultation. ...
... It predominantly occurs in women. 5 Imaging exams such as ultrasound, 10 magnetic resonance, 11 and computed tomography 12 can confirm the diagnosis. Recently, Amato et al. 13 published methodology for individual screening for lipedema using a selfadministered questionnaire that showed excellent diagnostic accuracy, 14 making it possible to estimate the prevalence of lipedema using Brazilian census data. ...
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Background Lipedema is characterized as an abnormal deposition of fat in the buttocks and legs bilaterally that may be accompanied by swelling, pain, and tenderness. It is still often confused with more frequent conditions such as obesity and lymphedema. The estimated prevalence in Europe varies between 0.06% and 39%. Objectives To evaluate the prevalence of lipedema and identify health factors related to it in the Brazilian population. Methods Administration of a previously validated online screening questionnaire to a representative sample of the general population. The questionnaire was distributed and administered to anonymous volunteers representing the general Brazilian population using software designed for population analyses. Results 253 women answered the questionnaire, 12.3 ± 4% (Confidence Interval [CI] 95%) of whom presented symptoms compatible with a high probability of being diagnosed with lipedema. Furthermore, anxiety, depression, hypertension, and anemia were also correlated with a high probability of the diagnosis. Conclusions The estimated prevalence of lipedema in the population of Brazilian women is 12.3%.
... Aufgrund der Hypothese, dass auch beim Lipödem pround antiinflammatorische Faktoren die Symptome beeinflussen können, fokussieren verschiedene Autoren die Bekämpfung der Inflammation und empfehlen eine antiinflammatorische und/oder ketogene Ernährung. 63,[73][74][75][76][77][78][79] In mehreren Fallserien und Studien zeigte sich im Vergleich zu anderen Ernährungsformen eine Überlegenheit der ketogenen Ernährung hinsichtlich der Reduktion lipödemtypischer Beschwerden. 63,[78][79][80][81][82] Stellenwert der bariatrischen Therapie in der Therapie von Patientinnen mit Lipödem Bei einem BMI > 40 kg/m 2 und einer WHtR über 0,55 wird zu kritischer Indikationsstellung geraten. ...
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Zusammenfassung Die vorliegende S2k‐Leitlinie wurde auf Initiative und unter Federführung der Deutschen Gesellschaft für Phlebologie und Lymphologie (DGPL) e.V. erstellt. Ihr Ziel ist es, die Diagnostik und Therapie des Lipödems zu optimieren. Sie soll die unterschiedlichen Herangehensweisen der jeweiligen Berufsgruppen konsensbasiert zusammenführen und damit ein Grundkonzept für die bestmögliche Behandlung von Patientinnen mit Lipödem bieten. Insgesamt wurden 60 Empfehlungen zu den Themenbereichen Diagnostik, konservative und chirurgische Therapieoptionen sowie psychosoziale Faktoren und Selbstmanagement formuliert und abgestimmt. Die Leitlinie soll den aktuellen Stand der Wissenschaft widerspiegeln und als Dokument für Diagnose‐ und Therapieempfehlungen bei Patientinnen mit Lipödem breite Anwendung finden. Insbesondere nimmt die Leitlinie Stellung zu Diagnosekriterien, Differenzialdiagnosen und koinzidenten Krankheitsbildern sowie deren Einfluss auf Diagnose und Therapie, sinnvollen Therapiemaßnahmen und Möglichkeiten des Selbstmanagements. Die Leitlinie Lipödem fasst die aktuelle nationale und internationale Evidenz sowie den deutschen Expertenkonsens zusammen und leitet daraus Empfehlungen ab, die zu bestmöglicher Behandlung von Patientinnen mit Lipödem führen sollen. Die Empfehlungen verstehen sich als Orientierungshilfe im Sinne von Handlungs‐ und Entscheidungskorridoren, von denen in begründeten Fällen abgewichen werden kann.
... 73 Based on the hypothesis that pro-and anti-inflammatory factors may influence the symptoms in lipedema, various authors focus on the treatment of inflammation and recommend an anti-inflammatory and/or ketogenic diet. 63,[73][74][75][76][77][78][79] In several case series and studies, ketogenic diet showed superiority over other forms of nutrition with respect to reduction of symptoms typical of lipedema. 63,[78][79][80][81][82] Significance of bariatric therapy for the treatment of patients with lipedema Recommendation 11.1 ...
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This S2k guideline was developed on the initiative and under the German Society of Phlebology and Lymphology (DGPL) leadership. The guideline aims to optimize the diagnosis and treatment of lipedema. It is intended to bring together the different approaches of the respective professional groups in a consensus‐based manner and thus offer a basic concept for the best possible treatment of patients with lipedema. Sixty recommendations were formulated and agreed on in diagnostics, conservative and surgical treatment options, psychosocial factors and self‐management. The guideline is intended to reflect the current scientific knowledge and be widely used for diagnosis and treatment recommendations for patients with lipedema. In particular, the guideline comments on diagnostic criteria, differential diagnoses, and coinciding clinical pictures, their influence on diagnosis and treatment, sensible treatment measures, and self‐management options. The lipedema guideline summarizes the current national and international evidence and the German expert consensus and derives recommendations for the best treatment for patients with lipedema. The recommendations in the guideline are intended as an orientation aid in the sense of action and decision‐making corridors from which deviations are possible in justified cases.
... 8 Partial improvement of lipedema symptoms doing a lymphedema treatment strategy could happen because of lymphatic vasculature dysfunction caused by lipedema inflammation. 9 In our view, we would be treating just the consequence of the problem, not the cause, 3 and the disease would continue to advance. ...
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Background: Lipedema is characterized as an abnormal deposition of fat in the buttocks and legs bilaterally that may be accompanied by swelling, pain, and tenderness. It is still often confused with more frequent conditions such as obesity and lymphedema. The estimated prevalence in Europe varies between 0.06% and 39%. Objectives: To evaluate the prevalence of lipedema and identify health factors related to it in the Brazilian population. Methods: Administration of a previously validated online screening questionnaire to a representative sample of the general population. The questionnaire was distributed and administered to anonymous volunteers representing the general Brazilian population using software designed for population analyses. Results: 253 women answered the questionnaire, 12.3 ± 4% (Confidence Interval [CI] 95%) of whom presented symptoms compatible with a high probability of being diagnosed with lipedema. Furthermore, anxiety, depression, hypertension, and anemia were also correlated with a high probability of the diagnosis. Conclusions: The estimated prevalence of lipedema in the population of Brazilian women is 12.3%.
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Case series Patients: Femal, 39-year-old • Femal, 64-year-old • Femal, 52-year-old • Femal, 34-year-old • Femal, 90-year-old Final Diagnosis: Lipedema Symptoms: Edema • pain Medication: — Clinical Procedure: Medications Specialty: Endocrinology and Metabolic Objective Unusual clinical course Background Lipedema is a chronic progressive disease characterized by the abnormal accumulation of fat in the subcutaneous region. Both medical and surgical treatments have been described in international guidelines; however, the current literature is biased toward promoting liposuction as the primary treatment of lipedema, and this can lead to the misapprehension that liposuction is the only form of definitive treatment. Case Reports In the present study, we report 5 cases at various stages of the evolution of lipedema, all with different therapeutic objectives. Case 1 reported having persistent bruising and pain, case 2 reported pain and fat deposition, case 3 reported night cramps and discomfort, case 4 reported leg thickening, and case 5 reported redness in the legs. All of were diagnosed with lipedema in different evolution stages. Our purpose was to demonstrate the possibility of non-surgical therapy, as well as to improve signs and symptoms of lipedema, using the QuASiL questionnaire and measuring changes in volumes and proportions. Good aesthetic outcomes improve both social and psychological status. Conclusions Currently, there are many described therapies available for lipedema. Liposuction surgery for lipedema should be considered one possible tool. Treatment objectives can be different for each patient. It is imperative to understand each patient’s needs in order to offer the best therapy attainable that meets patient requirements and induces a better quality of life. Non-surgical treatment of lipedema is feasible in selected cases, and it can meet the criteria for achieving selected clinical objectives.
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Contexto O lipedema é muito subdiagnosticado e faltam ferramentas auxiliares diagnósticas de baixo custo. Baseado em um questionário de avaliação sintomática, criamos e validamos um questionário de rastreamento do lipedema. Objetivos Os objetivos do trabalho foram a identificação de perguntas clínicas relevantes, a elaboração de questionário de rastreamento e a criação de modelo de predição do lipedema. Métodos Um questionário simplificado foi criado e aplicado em um grupo de pacientes com e sem lipedema, sendo avaliada a probabilidade de acerto no diagnóstico. Resultados Os 109 pacientes que responderam ao questionário eram do sexo feminino e as questões foram compreendidas. O modelo preditivo com perguntas individuais mostrou excelente probabilidade de acerto, de 91,2%, e o modelo preditivo com somatória de pontos também teve boa probabilidade de acerto, de 86,15%. Conclusões O questionário de rastreamento do lipedema é um instrumento prático, de fácil e rápida aplicação, que pode ser utilizado em nossa população para a identificação de possíveis pacientes com lipedema, aumentando o nível de suspeição no momento da anamnese e exame físico.
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Purpose The aim of this narrative review of the literature was to evaluate and summarize the current literature regarding the effect of lipedema on health-related quality of life (HRQOL) and psychological status. Methods The authors collected articles through a search into Medline, Embase, Scopus, Web of Science, Physiotherapy Evidence Database (PEDro), and the Cochrane Review. Search terms used included “Lipoedema,” “Lipedema,” “psychological status,” “Quality of life,” “Health related quality of life,” and “HRQOL.” Results A total of four observational studies were evaluated. The included studies were moderate-quality according to the Newcastle–Ottawa Scale. Three of the included studies demonstrated deterioration of HRQOL and psychological status in patients with lipedema. These studies also identify that pain and tenderness are a more common and dominant characteristic. Conclusion Future studies should establish a specific approach to treat and manage lipedema symptoms. Based on this narrative review of the literature findings, we recommended for the health care provider to pay more attention to HRQOL and psychological status. Moreover, validated and adapted measures of HRQOL and psychological status for patients with lipedema are required. Level of evidence Level V, narrative review.
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Introduction: The systemic inflammatory response has been proven to have a prognostic value. There are two methods of assessing the systemic inflammatory response composite ratios (R) and cumulative scores (S). The aim of this study was to compare the prognostic value of ratios and scores in patients undergoing surgery for colon cancer. Methods: Patients were identified prospectively in a single surgical unit. Preoperative neutrophil (N), lymphocyte (L), monocyte (M) and platelet (P) counts, CRP (C) and albumin (A) levels were recorded. The relationship between composite ratios neutrophil-lymphocyte ratio (NLR), platelet-lymphocyte ratio (PLR), lymphocyte-monocyte ratio (LMR), C-reactive protein albumin ratio (CAR) and the cumulative scores neutrophil- lymphocyte score (NLS), platelet-lymphocyte score (PLS), lymphocyte-monocyte score (LMS), neutrophil- platelet score (NPS), modified Glasgow prognostic score (mGPS) and clinicopathological characteristics, cancer-specific survival (CSS) and overall survival (OS), were examined. Results: A total of 801 patients were examined. When adjusted for tumour node metastasis (TNM) stage, NLR >5 (p < 0.001), NLS (p < 0.01), PLS (p < 0.001), LMR <2.4 (p < 0.001), LMS (p < 0.001), NPS (p < 0.001), CAR >0.22 (p < 0.001) and mGPS (p < 0.001) were significantly associated with CSS. In patients undergoing elective surgery (n = 689), the majority of the composite ratios/scores correlated with age (p < 0.01), BMI (p < 0.01), T stage (p < 0.01), venous invasion (p < 0.01) and peritoneal involvement (p < 0.01). When NPS (myeloid) and mGPS (liver) were directly compared, their relationship with CSS and OS was similar. Conclusions: Both composite ratios and cumulative scores had prognostic value, independent of TNM stage, in patients with colon cancer. However, cumulative scores, based on normal reference ranges, are simpler and more consistent for clinical use.
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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.