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Introdução: O lipedema, recentemente introduzido na 11ª revisão da Classificação Internacional de Doenças. Estima-se que metade das pessoas acometidas pelo lipedema, estejam em sobrepeso ou com obesidade, sendo uma associação de comorbidades que dificulta o diagnóstico. O diagnóstico errôneo é preocupante, pois atrasa o tratamento da doença permitindo a sua progressão. Objetivo: Trata-se de uma revisão integrativa da literatura (RIL) com objetivo de analisar o que se tem construído em pesquisas anteriores relacionadas ao lipedema. Metodologia: A busca dos artigos científicos foram os disponibilizados nos Periódicos da Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES). Os artigos constassem os Descritores em Ciências da Saúde (DeCS) com as palavras: lipedema and procedimento and cirurgia and mulher, e que fossem revisados por pares, nas línguas inglesa e portuguesa. a partir da aplicação dos critérios previamente definidos O período de busca foi realizado nos meses de maio a agosto de 2023. Resultados e Discussão: Foram elegíveis 12 artigos. A análise dos artigos encontrados possibilitou a evidente necessidade de conhecimento e clareza no diagnóstico do lipedema. As mulheres são as mais afetadas pela doença. A área afetada pode incluir os quadris, as pernas bilateral e simetricamente. E uma das principais características distintivas do lipedema é a preservação dos pés, o que pode criar um degrau distinto no tornozelo. Assim, a importância do diagnóstico diferencial. O procedimento cirúrgico como tratamento apresentou melhores resultados. Entretanto, a associação de todos os tratamentos sobressaiu na qualidade de melhora dos aspectos do lipedema. Conclusão: Essa revisão apontou para o complexo  diagnóstico do lipedema.  Esse assunto persiste como um desafio e deve instigar mais pesquisadores na busca para um diagnóstico preciso, opções de tratamento e até mesmo no sentido de prevenção para satisfazer a população assistida, no caso a maioria são as mulheres.  A melhor opção indicada pelos estudos analisados foi o tratamento cirúrgico realizado pela lipoaspiração.

BACKGROUND: Lipedema, a complex and enigmatic adipose tissue disorder, remains poorly understood despite its significant impact on the patients' quality of life. Genetic investigations have uncovered potential contributors to its pathogenesis, including somatic mutations, which are nonheritable genetic alterations that can play a pivotal role in the development of this disease. AIM: This review aims to elucidate the role of somatic mutations in the etiology of lipedema by examining their implications in adipose tissue biology, inflammation, and metabolic dysfunction. RESULTS: Studies focusing on leukocyte clones, genetic alterations like TET2 and DNMT3A, and the intricate interplay between adipose tissue and other organs have shed light on the underlying mechanisms driving lipedema. From the study of the scientific literature, mutations to genes correlated to three main pathways could be involved in the somatic development of lipedema: genes related to mitochondrial activity, genes related to localized disorders of subcutaneous adipose tissue, and genes of leukocyte clones. CONCLUSIONS: The insights gained from these diverse studies converge to highlight the complex genetic underpinnings of lipedema and offer potential avenues for therapeutic interventions targeting somatic mutations to alleviate the burden of this condition on affected individuals.

PURPOSE OF REVIEW: This review aims to provide an overview of the current evidence on the efficacy, also considering the anti-inflammatory properties and safety of very low-calorie ketogenic diet (VLCKD) as a potential treatment for lipedema, particularly in the context of obesity. RECENT FINDINGS: Lipedema is a chronic disease characterized by abnormal and painful fat buildup on the legs and/or arms. It is often misdiagnosed as obesity or lymphedema. However, although lipedema and obesity can coexist, unlike obesity, lipedema usually affects the legs and thighs without affecting the feet or hands, and the abnormal deposition of adipose tissue in lipedema is painful. The current lifestyle interventions are often unsuccessful in the management of lipedema. There is no consensus on the most effective nutritional approach for managing lipedema. Recent studies have suggested that VLCKD may be an effective treatment for lipedema, demonstrating that it is also superior to other nutritional approaches such as Mediterranean diet or intermittent fasting. Lipedema is a chronic and debilitating disease characterized by abnormal and painful accumulation of adipose tissue in the legs. VLCKD has been shown to be an effective treatment for lipedema, especially in the context of obesity, due to its anti-inflammatory properties. However, further research is needed to determine the long-term safety and efficacy of VLCKD as a treatment for lipedema.

BACKGROUND: Diagnosing lipedema remains a challenge due to its heterogeneous presentation, co-existing diseases, and the lack of objective diagnostic imaging. OBJECTIVE: This systematic review aims to outline the currently available diagnostic imaging methods to characterize lipedema in the legs along with their diagnostic performance. METHODS: PubMed, Embase, Google Scholar, Scopus, and Web of Science were searched. The quality assessment of diagnostic accuracy studies (QUADAS) tool was used for quality assessment. RESULTS: Thirty-two studies describing a total of 1154 patients with lipedema were included for final analysis. Features for lipedema have been defined using ultrasound (increased subcutaneous adipose tissue), lymphoscintigraphy (slowing of the lymphatic flow and a frequent asymmetry between the lower extremities), computed tomography (symmetrical bilateral soft tissue enlargement without either skin thickening or subcutaneous edema), magnetic resonance imaging (increased subcutaneous adipose tissue), MR lymphangiography (enlarged lymphatic vessels up to a diameter of 2 mm), and dual-energy X-ray absorptiometry (fat mass in the legs adjusted for body mass index (BMI) ≥ 0.46 or fat mass in the legs adjusted for total fat mass ≥ 0.384). CONCLUSION: The diagnostic performance of currently available imaging modalities for assessing lipedema is limited. Prospective studies are needed to evaluate and compare the diagnostic performance of each imaging modality. Imaging techniques focusing on the pathogenesis of the disease are needed.

Lymphedema and specifically cancer-related lymphedema is not the main focus for both patients and physicians dealing with cancer. Its etiology is an unfortunate complication of cancer treatment. Although lymphedema treatments have gained an appreciable consensus, many practitioners have developed and prefer their own specific protocols and this is especially true for conventional (manual) versus surgical treatments. This collection of presentations explores the incidence and genetics of cancer-related lymphedema, early detection and monitoring techniques, both conventional and operative treatment options, and the importance and role of exercise for patients with cancer-related lymphedema. These assembled presentations provide valuable insights into the challenges and opportunities presented by cancer-related lymphedema including the latest research, treatments, and exercises available to improve patient outcomes and quality of life.

Lipohyperplasia dolorosa (LiDo) is a genetic, painful fat tissue distribution disorder with lymphological high-volume transport insufficiency. It often has negative effects on the psychological well-being of affected female adolescents and adults. Similar in appearance to the development of obesity, the patients experience similar negative reactions in their families, partners and friends. The development of the LiDo usually occurs in adolescence or following pregnancy and represents a considerable psychological burden in central phases of narcissistic development. These psychological impairments caused by LiDo are long-term companions and influence interpersonal relationships. Three case vignettes serve for clarification. In the first case, the LiDo seems to be "grafted" onto a neurotic conflict, which intensifies the acute and chronic pain of the person affected. In the second case, the affected person shows defense mechanisms in contact, which are evidence of a high level of stress and require considerable sensitivity by the person's social circle during interactions. In the third case, after intensively addressing various aspects of the disease, the person received medical treatment from a specialist and underwent several surgeries. The positive effects on physical and psychological well-being are stabilized by psychological support. Seen as an option, those affected can decide for or against surgical treatment. As a consequence of the treatment, the previously rejected extremities become more integrated, arms and legs fit back into the person's own physical image of the body. This positive change also extends to the intrapsychic self-image of the body.

Background Lipedema is a loose connective tissue disease characterized by a disproportionate accumulation of adipose tissue in the limbs of women. Despite its incidence of 10-20%, lipedema is often underdiagnosed and misdiagnosed. Objectives This review aims to outline current available evidence regarding this enigmatic syndrome and gives a synopsis on the subjects that are still unknown. Materials and Methods PubMed and Embase searches were conducted to identify relevant articles on lipedema pathophysiology, clinical presentation, diagnosis and treatment. Results Lipedema can be considered a disease of the adipocytes or a circulatory disorder of the lymphatics. The relationship between lymphatics and adipose tissue remains controversial. The clinical distinction between lipedema, lymphedema, phlebolymphedema and lipolymphedema can be difficult. Diagnoses often coexist, further complicating the diagnosis of lipedema, which is currently made on clinical grounds alone. The value of diagnostic imaging studies is unclear. Liposuction appears to be an effective treatment and significantly improves symptoms. Conclusion Diagnosing lipedema remains a challenge due to its heterogeneous presentation, co-existing diseases, and the lack of objective diagnostic imaging. Further directions for research include the effect of excess skin resection surgery on lymphatic drainage.

A large and growing body of research suggests that the skin plays an important role in regulating total body sodium, challenging traditional models of sodium homeostasis that focused exclusively on blood pressure and the kidney. In addition, skin sodium may help to prevent water loss and facilitate macrophage-driven antimicrobial host defense, but may also trigger immune dysregulation via upregulation of pro-inflammatory markers and downregulation of anti-inflammatory processes. We performed a systematic search of PubMed for published literature on skin sodium and disease outcomes and found that skin sodium concentration is increased in patients with cardiometabolic conditions including hypertension, diabetes, and end-stage renal disease; autoimmune conditions including multiple sclerosis and systemic sclerosis; and dermatologic conditions including atopic dermatitis, psoriasis, and lipedema. Several patient characteristics are associated with increased skin sodium concentration including older age and male sex. While animal evidence suggests that increased salt intake results in higher skin sodium levels, there are conflicting results from small trials in humans. Additionally, limited data suggest that pharmaceuticals such as diuretics and SGLT-2 inhibitors approved for diabetes, as well as hemodialysis may reduce skin sodium levels. In summary, emerging research supports an important role for skin sodium in physiologic processes related to osmoregulation and immunity. With the advent of new non-invasive MRI measurement techniques and continued research on skin sodium, it may emerge as a marker of immune-mediated disease activity or a potential therapeutic target.

Lymphangiogenesis is the mechanism by which the lymphatic system develops and expands new vessels facilitating fluid drainage and immune cell trafficking. Models to study lymphangiogenesis are necessary for a better understanding of the underlying mechanisms and to identify or test new therapeutic agents that target lymphangiogenesis. Across the lymphatic literature, multiple models have been developed to study lymphangiogenesis in vitro and in vivo. In vitro, lymphangiogenesis can be modeled with varying complexity, from monolayers to hydrogels to explants, with common metrics for characterizing proliferation, migration, and sprouting of lymphatic endothelial cells (LECs) and vessels. In comparison, in vivo models of lymphangiogenesis often use genetically modified zebrafish and mice, with in situ mouse models in the ear, cornea, hind leg, and tail. In vivo metrics, such as activation of LECs, number of new lymphatic vessels, and sprouting, mirror those most used in vitro, with the addition of lymphatic vessel hyperplasia and drainage. The impacts of lymphangiogenesis vary by context of tissue and pathology. Therapeutic targeting of lymphangiogenesis can have paradoxical effects depending on the pathology including lymphedema, cancer, organ transplant, and inflammation. In this review, we describe and compare lymphangiogenic outcomes and metrics between in vitro and in vivo studies, specifically reviewing only those publications in which both testing formats are used. We find that in vitro studies correlate well with in vivo in wound healing and development, but not in the reproductive tract or the complex tumor microenvironment. Considerations for improving in vitro models are to increase complexity with perfusable microfluidic devices, co-cultures with tissue-specific support cells, the inclusion of fluid flow, and pairing in vitro models of differing complexities. We believe that these changes would strengthen the correlation between in vitro and in vivo outcomes, giving more insight into lymphangiogenesis in healthy and pathological states.

The disease "Lipedema", which has been known since 1940, is increasingly better understood. Dimpled edema in particular is not significant in women with fat distribution disorders on the arms and legs. These and other scientific findings are "work in progress" with the aim of renaming the disease. A "proper name" is "Lipohyperplasia dolorosa" (LiDo). With LiDo, the increase in volume is genetically fixed, but the pain is dynamically progressive. A LiDo must be distinguished from other symmetrical, painless fat distribution disorders on the arms and legs at first sight and after palpatory examination, especially from the occasionally coincident obesity. Obesity is never comorbid, but often coincident with LiDo. Although physical activity and a change in diet can reduce obesity, they cannot eliminate the disproportionate increase in fat tissue on the extremities that is exclusively caused by LiDo. In LiDo patients coincident with obesity, gastric surgery has no effect on the obligatory pain. There are both conservative and surgical treatment options for LiDo. A procedure that has been established since 1997 is surgical treatment using lymphological liposculpture. As part of this operation, large wounds are created under the skin, which, according to the "Rules of Nine" when treating both arms in one session and the suction of the legs in 2 sessions per operation, correspond to an area of ​​18% of the body surface. However, with adequate postoperative management and the administration of antibiotics and antithrombotics, local complications associated with the operation are rare. The most important result of consistent surgical treatment of lipohyperplasia dolorosa is the impact on quality of life: "It ruined her life" becomes "It improves her life".

As overweight and obesity rates have increased worldwide, the prevalence of metabolic disorders has also grown. Due to the lack of physiologically relevant adipose tissue platforms, research in adipose tissue biology has relied on animal models, leading to false conclusions on pathophysiological mechanisms and therapeutic efficacy. Despite the urgent need for an adipose tissue model, it is still extremely difficult to cultivate mature adipocytes and recapitulate multi-cellular interactions in adipose tissue in vitro. For this reason, adipose tissue modeling requires new technologies that allow better culture conditions for adipocytes and contain a complex network of microenvironments. Herein, we discuss recent technologies, including 3-dimensional (3D) adipocyte spheroids, biomaterial-based 3D culture, 3D bioprinting, and microphysiological systems, which may offer new opportunities to discover drugs targeting adipose tissue.

Steroid hormones synchronize a variety of functions throughout all stages of life. Importantly, steroid hormone-transforming enzymes are ultimately responsible for the regulation of these potent signaling molecules. Germline mutations that cause dysfunction in these enzymes cause a variety of endocrine disorders. Mutations in SRD5A2, HSD17B3, and HSD3B2 genes that lead to disordered sexual development, salt wasting, and other severe disorders provide a glimpse of the impacts of mutations in steroid hormone transforming enzymes. In a departure from these established examples, this review examines disease-associated germline coding mutations in steroid-transforming members of the human aldo-keto reductase (AKR) superfamily. We consider two main categories of missense mutations: those resulting from nonsynonymous single nucleotide polymorphisms (nsSNPs) and cases resulting from familial inherited base pair substitutions. We found mutations in human AKR1C genes that disrupt androgen metabolism, which can affect male sexual development and exacerbate prostate cancer and polycystic ovary syndrome (PCOS). Others may be disease causal in the AKR1D1 gene that is responsible for bile acid deficiency. However, given the extensive roles of AKRs in steroid metabolism, we predict that with expanding publicly available data and analysis tools, there is still much to be uncovered regarding germline AKR mutations in disease.

When studying the current literature, one might get the impression that lipedema is a “modern” disease, with increasing incidence and augmenting prevalence throughout Western countries during the last decade. However, a quick look into older textbooks shows that disproportionate accumulation of fat in female bodies has long been known without being recognized as an independent disease. Nevertheless, it was not until 1940 that Allen and Hines described a “syndrome characterized by fat legs and orthostatic edema” in a seminal publication. The mere awareness that people who have lipedema are not just overweight but suffer from a yet poorly defined pathological condition, may be considered a decisive leap forward in the understanding of lipedema. A number of comprehensive publications have since dealt with the clinical presentation of lipedema and have provided the first clues towards the potential pathological mechanisms underlying its initiation and progression. Nevertheless, despite all effort that has been undertaken to unravel lipedema pathology, many questions have remained unanswered. What can be deduced with certainty from all experimental and medical evidence available so far is that lipedema is neither a cosmetic problem nor is it a problem of lifestyle but should be accepted as a serious disease with yet undetermined genetic background, which makes women’s lives unbearable from both a physical and psychological point of view. To date, results from clinical inspections have led to the categorization of various types and stages of lipedema, describing how the extremities are affected and evaluating its progression, as demonstrated by skin alterations, adipose tissue volume increase and physical and everyday-behavioral impediments. There is accumulating evidence showing that advanced stages of lipedema are usually accompanied by excessive weight or obesity. Thus, it is not unreasonable to assume that the progression of lipedema is largely driven by weight gain and the pathological alterations associated with it. Similarly, secondary lymphedema is frequently found in lipedema patients at advanced stages. Needless to say, both conditions considerably blur the clinical presentation of lipedema, making diagnosis difficult and scientific research challenging. The present literature review will focus on lipedema research, based on evidence fromex vivo and in vitro data, which has accumulated throughout the last few decades. We will also open the discussion as to whether the currently used categorization of lipedema stages is still sufficient and up-to-date for the accurate description of this enigmatic disease, whose name, strangely enough, does not match its pathologic correlate.

Lipoedema is a painful non-pitting diffuse “fatty” swelling, usually confined to the legs, that occurs mainly in women. This scoping review aimed to provide an overview of the available research on the functioning of people with lipoedema, according to the International Classification of Functioning, Disability and Health (ICF) framework. Relevant publications and gray literature were retrieved until October 2022. The results sections of each publication were organized using a thematic framework approach. All included studies reported at least one outcome fitting within the domains of body functions and body structures, with most studies focusing on the categories of “sensation of pain”, “immunological system functions”, and “weight maintenance functions”. The ICF domains of activities and participation and environmental factors were mentioned in a small number of the included studies (17 and 13%, respectively), while the domain of personal factors was studied in half of the included studies. In conclusion, the emphasis of lipoedema research is on its description from a disorder-oriented point of view in the form of body functions and body structures, with a lack of information about the other domains of functioning.

Enhancing our understanding of lymphatic anatomy from the microscopic to the anatomical scale is essential to discern how the structure and function of the lymphatic system interacts with different tissues and organs within the body and contributes to health and disease. The knowledge of molecular aspects of the lymphatic network is fundamental to understand the mechanisms of disease progression and prevention. Recent advances in mapping components of the lymphatic system using state of the art single cell technologies, the identification of novel biomarkers, new clinical imaging efforts, and computational tools which attempt to identify connections between these diverse technologies hold the potential to catalyze new strategies to address lymphatic diseases such as lymphedema and lipedema. This manuscript summarizes current knowledge of the lymphatic system and identifies prevailing challenges and opportunities to advance the field of lymphatic research as discussed by the experts in the workshop.

Often regarded as the forgotten second circulatory system, the lymphatic system is critical in working with the venous and arterial system to maintain fluid equilibrium, circulate cells and signaling molecules in the immune system, and transport fat molecules and nutrients. When there is an alteration in the flow of lymphatic fluid, lymphedema is the result, usually manifesting as significant swelling of a particular region of the body with protein-rich fluid accumulation in the interstitial spaces. The result of this disease ranges from psychological disturbance to significant disability and morbidity, even leading to aggressive malignancy. The prevalence of lymphedema is in the hundreds of millions worldwide; however, it is routinely under-diagnosed and under-recognized as a disease, especially in the United States. Treatment rates for lymphedema are variable, ranging from low rates of treatment in non-cancer etiologies, to high treatment rates seen in breast cancer related etiologies. Furthermore, treatment modalities are equally notorious for being unsuccessful, either as a result of non-compliance or lack of efficacious therapeutic techniques, or a combination of both. There is no cure for lymphedema and treatment mainly focuses on minimizing limb swelling, maintaining functionality, and preventative treatments to reduce complications.

Lipedema is a relatively common, unrecognized connective tissue disorder. It affects about 11% of women across the globe. In lipedema, there is symmetrical abnormal deposition of fat especially in the lower extremities. Lipedema can be accompanied with pain, spider veins and easy bruising. Lipedema is rare in men. The disease is progressive in nature. Trigger points for lipedema appear to be puberty, pregnancy and menopause. The disease affects various aspects of the connective tissues like lymphatics, veins, skin, ligaments and even joints as it progresses. Oestrogen and progesterone abnormalities seem to have a role in the progression of the condition. Lipedema also appears to have a genetic predisposition. Lipedema is classified into 4 stages depending on the degree and type of abnormal fat deposition. The severe form can be confused with Dercum’s disease. Depending on the site of deposition of abnormal fat, lipedema is also classified into 5 different types. Lipedema is often confused to be obesity or lymphedema and treated accordingly. Treatments are based on the stages and types of lipedema. Lipedema is extremely difficult to manage with diet, exercises and bariatric surgery alone. All treatments are focused around improving the quality of life, improving physical abilities and appearances.