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Lipedema is a symmetrical disorder of the subcutaneous adipose tissue that affects almost exclusively women in postpubescent age. The trinity of disfiguring hyperplastic adipose tissue of the proximal extremities, increasing pain over time and bruising is characteristic. Lymphedema can occur in both sexes and is differentiated into primary and secondary subtypes. Symmetry is not a prerequisite for diagnosis. Characteristic for lymphedema is the disproportion between lymphatic fluid and the capacity of lymphatic vessels. The most distal body parts are always more severely affected than proximal parts. In the initial phases lymphedema is painless but in advanced stages tension pain can occur. The combination of both disorders has been described; however, lipedema is not responsible for subsequent lymphedema in contrast to central obesity, which significantly increases the risk of lymphedema. The differential diagnosis is of utmost importance for a meaningful management.
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Under the direction of the German Society of Phlebology (Deutsche Gesellschaft für Phlebologie) and in cooperation with other specialist associations, the S1 guideline on intermittent pneumatic compression (IPC) was adopted in January 2018. It replaces the previous guideline from March 2005. The aim of the guideline is to optimize the indication and therapeutic use of IPC in vascular diseases and edema. An extensive literature search of MEDLINE, existing guidelines, and work relevant to the topic was performed. In view of the often methodologically weak study quality with often small numbers of cases and heterogeneous treatment protocols, recommendations can often only be derived from the available data using good clinical practice/expert consensus. Intermittent pneumatic compression is used for thromboembolism prophylaxis, decongestive therapy for edema, and to positively influence arterial and venous circulation to improve clinical symptoms and accelerate ulcer healing in both the outpatient and inpatient care setting. The therapy regimens and devices used depend on the indication and target location. They can be used as outpatient and inpatient devices as well as at home for long-term indications. A target indication is thrombosis prophylaxis. IPC should be used in severe chronic venous insufficiency (stages C4b to C6), in extremity lymphedema as an add-on therapy and in peripheral arterial occlusive disease (PAOD) with stable intermittent claudication or critical ischemia. IPC can be used in post-traumatic edema, therapy-resistant venous edema, lipedema and hemiplegia with sensory deficits and edema. Absolute and relative contraindications to IPC must be taken into account and risks considered and avoided as far as possible. Adverse events are extremely rare if IPC is used correctly. If the indication and application are correct-also as an add-on therapy-it is a safe and effective treatment method, especially for the treatment of the described vascular diseases and edema as well as thrombosis prophylaxis.
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Lipedema is a little-known alteration or disorder in the distribution of body fat, which affects almost exclusively women and primarily involves the lower extremities. Epidemiological data are currently scarce and not helpful to determine the exact incidence of lipedema in the general population; its etiology and physiopathology are not clear enough; its diagnosis is basically clinical, since there are no specific diagnostic tests or enough scientific evidence to support it. However, its clinical manifestations imply deterioration in patients’ quality of life due to the physical, psychological and social impact it entails. Since about 70% of cases are associated with pain in extremities, these are usually referred to phlebology and lymphology specialists. Treatments to control the edema are not usually successful in reducing the volume of the extremities. This is in addition to determinants of fashion from the 21st century that demand most women to have slender bodies, while the reality is that overweight and obesity are alarmingly increasing. About 80% of obese patients do not admit to have excess weight, complicating its early diagnosis and prevention of its evolution and, consequently, delaying treatment in patients with this disease. Due to the clinical presentation and symptoms associated with lipedema, patients are often misdiagnosed with lymphedema, obesity, lipodystrophies or chronic venous insufficiency, and therefore they are not correctly and effectively treated; in the best case scenario, they are administered symptomatic treatments. In the absence of unified criteria for lipedema, and given the national and international controversy surrounding the term, the Spanish Association of Lymphedema and Lipedema (AEL) has put together a multidisciplinary working group of health professionals from different fields involved in the diagnosis and treatment of this disorder to draft this Consensus Document. The aim was to answer multiple questions using the documentary evidence and clinical experience available to date. Is the currently described physiopathology enough to explain lipedema? Is it a progressive alteration? Does it always get worse? When you have lipedema, is the progressive increase in body fat percentage normal? If the main symptom is not the edema: Is manual lymphatic drainage an essential tool for the treatment? Is it correct to prescribe compression garments in all cases? What are the most effective treatments? This working group included the participation of: The Spanish Association of Lymphedema and Lipedema (AEL), the Spanish Chapter of Phlebology and Lymphology (CEFyL) from the Spanish Society of Angiology and Vascular Surgery (SEACV), the Spanish Society of Aesthetic Medicine (SEME), the Spanish Society of Plastic, Reconstructive and Aesthetic Surgery (SECPRE), the Spanish Society of Dietetics and Food Science (SEDCA), the Complutense University of Madrid, and doctors specialized in Physical Medicine and Rehabilitation from the Spanish Society of Rehabilitation and Physical Medicine (SERMEF). This document includes several chapters on the definition and physiopathology of lipedema, its diagnostic methods, differential diagnosis, classification and treatment using physical, pharmacological and surgical means. It is hoped that it can help people with lipedema and health professionals caring for them. However, there is still a lot to learn about the etiopathogenesis, diagnosis and treatment of lipedema, so research must continue and be completed with epidemiological studies of its incidence and prevalence, always using an interdisciplinary approach.
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