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The four previous articles in this series addressed the myths and facts surrounding lipoedema. We have shown that there is no scientific evidence at all for the key statements made about lipoedema – which are published time and time again. The main result of this “misunderstanding” of lipoedema is a therapeutic concept that misses the mark. The patient’s real problems are overlooked.The national and especially the international response to the series, which can be read in both German and English, has been immense and has exceeded all our expectations. The numerous reactions to our articles make it clear that in other countries, too, the fallacies regarding lipoedema have led to an increasing discrepancy between the experience of healthcare workers and the perspective of patients and self-help groups, based on misinformation mostly generated by the medical profession.Parts 1 to 4 in this series of articles on the myths surrounding lipoedema have made it clear that we have to radically change the view of lipoedema that has been held for decades. Changing our perspective means getting away from the idea of “oedema in lipoedema” – and hence away from the dogma that decongestion is absolutely necessary – and towards the actual problems faced by our patients with lipoedema. Such a paradigm shift in a disease that has been described in the same way for decades cannot be left to individuals but must be put on a much broader footing. For this reason, the lead author of this series of articles invited renowned lipoedema experts from various European countries to discussions on the subject. Experts from seven different countries took part in the two European Lipoedema Forums, with the goal of establishing a consensus. The consensus reflects the experts’ shared view on the disease, having scrutinized the available literature, and having taken into account the many years of clinical practice with this particular patient group. Appropriate to the clinical complexity of lipoedema, participants from different specialties provided an interdisciplinary approach. Nearly all of the participants in the European Lipoedema Forum had already published work on lipoedema, had been involved in drawing up their national lipoedema guidelines, or were on the executive board of their respective specialty society.In this fifth and final part of our series on lipoedema, we will summarise the relevant findings of this consensus, emphasising the treatment of lipoedema as we now recommend it. As the next step, the actual consensus paper “European Best Practice of Lipoedema” will be issued as an international publication.Instead of looking at the treatment of oedema, the consensus paper will focus on treatment of the soft tissue pain, as well as the psychological vulnerability of patients with lipoedema. The relationship between pain perception and the patient’s mental health is recognised and dealt with specifically. The consensus also addresses the problem of self-acceptance, and this plays a prominent role in the new therapeutic concept. The treatment of obesity provides a further pillar of treatment. Obesity is recognised as being the most common comorbid condition by far and an important trigger of lipoedema. Bariatric surgery should therefore also be considered for patients with lipoedema who are morbidly obese. The expert group upgraded the importance of compression therapy and appropriate physical activity, as the demonstrated anti-inflammatory effects directly improve the patients’ symptoms. Patients will be provided with tools for personalised self-management in order to sustain sucessful treatment. Should conservative therapy fail to improve the symptoms, liposuction may be considered in strictly defined circumstances.The change in the view of lipoedema that we describe here brings the patients’ actual symptoms to the forefront. This approach allows us to focus on more comprehensive treatment that is not only more effective but also more sustainable than focusing on the removal of non-existent oedema.

Lokale Odeme finden sich als klinisches Symptom bei vielen Hautkrankheiten, zumeist im Rahmen von Entzundungen oder allergischen Reaktionen. Erkrankungen des Lymphgefassystems fuhren ebenfalls zu einem Odem mit spezifischen Veranderungen der Haut. Lymphodeme sind Folge primarer, genetischer Veranderungen, die auch im Rahmen von Syndromen auftreten konnen und auf eine fehlerhafte Ausbildung des Lymphgefassystems zuruckzufuhren sind. Sekundare Lymphodeme entstehen meist nach Operationen, Entzundungen oder chronischer venoser Insuffizienz (CVI). Das Mikrofiltrat, das im Gewebe bei Gesunden uber das lymphatische System abtransportiert wird, ist proteinreich und induziert eine Fibrosierung der Epidermis und Dermis. Klinische Zeichen sind neben dem Odem Pachydermie, Papillomatosis cutis lymphostatica, Hyperkeratosen, Lymphzysten, Lymphfisteln, Nageldystrophien und gelbe Nagel. Die Basistherapie besteht aus der komplexen physikalischen Entstauung (KPE: manuelle Lymphdrainage, Kompression, Bewegung/Sport, Hautpflege und Anleitung zum Selbstmanagement). Adjuvant kann die apparative intermittierende Kompression angewendet werden. Komorbiditaten, wie z. B. Adipositas und Diabetes mellitus, wirken aggravierend und sind unbedingt mit zu behandeln. Durch adaquate Therapie konnen Spatfolgen der Haut reduziert werden.

Under the direction of the German Society of Phlebology (Deutsche Gesellschaft fur 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.

The objective of the present S1 guidelines is to present current knowledge about dermatologically relevant diseases associated with localized dermal lymphostasis, thus facilitating their early detection, diagnostic workup, and targeted treatment. Whenever possible, treatment should be based on stage-appropriate and clearly defined algorithms. The numerous issues regarding differential diagnosis and treatment clinicians are confronted with in everyday clinical practice seem to warrant the publication of up-to-date guidelines. These guidelines focus on patients of all age groups and genders exhibiting skin lesions caused by dermal lymphostasis. Specific recommendations are provided with respect to the diagnosis and differential diagnosis of the various clinical manifestations. In this context, comorbid skin diseases such as atopic dermatitis, psoriasis, hidradenitis suppurativa, urticaria, and contact dermatitis will be highlighted, including their treatment and associated specific risks. Several other relevant current guidelines are referenced as regards the distinction from and treatment of common cofactors and comorbid conditions.

Das Ziel dieser S1-Leitlinie ist es, aktuelles Wissen uber dermatologisch relevante Krankheitsbilder bei lokal begrenzter dermaler Lymphostase an allen Lokalisationen des Hautorgans zu vermitteln, um diese fruhzeitig zu erkennen, diagnostisch zu sichern und gezielt zu behandeln. Wann immer moglich, sollte diese Therapie anhand klar definierter Algorithmen stadiengerecht erfolgen. Die im klinischen Alltag haufig auftauchenden differenzialdiagnostischen und therapeutischen Fragen lassen eine aktuelle Leitlinie notwendig erscheinen. Diese Leitlinie fokussiert auf Patienten jeglichen Alters und Geschlechts mit Hautveranderungen, die im kausalen Zusammenhang mit dermaler Lymphostase stehen. In konkreten Handlungsempfehlungen werden die Diagnostik und Differenzialdiagnostik der verschiedenen Manifestationsformen bei gleichzeitig auftretender dermatologischer Komorbiditat wie beispielsweise atopische Dermatitis, Psoriasis vulgaris, Acne inversa, Urtikaria, Kontaktekzeme sowie ihre Therapien unter Berucksichtigung besonderer Risiken veranschaulicht. Fur die Abgrenzung und Behandlung haufiger therapierelevanter Kofaktoren und Komorbiditat wird auf mehrere andere hierfur relevante aktuelle Leitlinien verwiesen.

Summary The present, revised guidelines on lipedema were developed under the auspices of and funded by the German Society of Phlebology (DGP). The recommendations are based on a systematic literature search and the consensus of eight medical societies and working groups. The guidelines contain recommendations with respect to diagnosis and management of lipedema. The diagnosis is established on the basis of medical history and clinical findings. Characteristically, there is a localized, symmetrical increase in subcutaneous adipose tissue in arms and legs that is in marked disproportion to the trunk. Other findings include edema, easy bruising, and increased tenderness. Further diagnostic tests are usually reserved for special cases that require additional workup. Lipedema is a chronic, progressive disorder marked by the individual variability and unpredictability of its clinical course. Treatment consists of four therapeutic mainstays that should be combined as necessary and address current clinical symptoms: complex physical therapy (manual lymphatic drainage, compression therapy, exercise therapy, and skin care), liposuction and plastic surgery, diet, and physical activity, as well as psychotherapy if necessary. Surgical procedures are indicated if – despite thorough conservative treatment – symptoms persist, or if there is progression of clinical findings and/or symptoms. If present, morbid obesity should be therapeutically addressed prior to liposuction.

Die vorliegende uberarbeitete Leitlinie zum Lipodem wurde unter der Federfuhrung der Deutschen Gesellschaft fur Phlebologie (DGP) erstellt und finanziert. Die Inhalte beruhen auf einer systematischen Literaturrecherche und dem Konsens von acht medizinischen Fachgesellschaften und Berufsverbanden. Die Leitlinie beinhaltet Empfehlungen zu Diagnostik und Therapie des Lipodems. Die Diagnose ist dabei auf der Basis von Anamnese und klinischem Befund zu stellen. Charakteristisch ist eine umschriebene, symmetrisch lokalisierte Vermehrung des Unterhautfettgewebes an den Extremitaten mit deutlicher Disproportion zum Stamm. Zusatzlich finden sich Odeme, Hamatomneigung und eine gesteigerte Schmerzhaftigkeit der betroffenen Korperabschnitte. Weitere apparative Untersuchungen sind bisher besonderen Fragestellungen vorbehalten. Die Erkrankung ist chronisch progredient mit individuell unterschiedlichem und nicht vorhersehbarem Verlauf. Die Therapie besteht aus vier Saulen, die individuell kombiniert und an das aktuelle Beschwerdebild angepasst werden sollten: komplexe physikalische Entstauungstherapie (manuelle Lymphdrainage, Kompressionstherapie, Bewegungstherapie, Hautpflege), Liposuktion und plastisch-chirurgische Interventionen, Ernahrung und korperliche Aktivitat sowie ggf. additive Psychotherapie. Operative Masnahmen sind insbesondere dann angezeigt, wenn trotz konsequent durchgefuhrter konservativer Therapie noch Beschwerden bestehen bzw. eine Progredienz des Befundes und/oder der Beschwerden auftritt. Eine begleitend zum Lipodem bestehende morbide Adipositas sollte vor einer Liposuktion therapeutisch angegangen werden.

Lymphoedema is a chronic disease affecting the patients' quality of life (QoL) especially with regard to mobility and daily activities. Lymphoedema cannot be cured but symptoms can effectively be improved by a comprehensive approach and the continuous use of compression therapy. An appropriate fitted compression garment is essential for compliance and thus a prerequisite for successful lymphoedema management. Compression garments with bulky seams are not only uncomfortable they also can lead to severe pressure marks or even reduce the mobility and function of the affected limb, which may negatively impact the already reduced QoL of lymphoedema patients. A prospective multicentre observational study was conducted to compare compression garments without and with a seam for hands and toes in 143 patients with lymphoedema. Product performance, lymphoedema symptoms, functionality of hands/toes, QoL and patient satisfaction were documented for the previously worn compression garment as well as for the 3D flat knitted JOBST® Elvarex Plus compression garment without a seam. The JOBST Elvarex Plus glove was perceived very positively by patients, who rated the mobility of the fingers, the adaptability to the shape of the hand/fingers, the fit of cuffs/welts, and the tactile sense of the fingers to be significantly better compared to the previous glove. Also pressure marks between fingers/toes and pressure marks due to cuffs/welts were significantly less frequent when wearing JOBST Elvarex Plus. Also pain, sensation of heaviness, swelling, tightness and dryness of skin were significantly improved when wearing JOBST Elvarex Plus gloves. Furthermore, 80% of the patients rated the wearing comfort of JOBST Elvarex Plus to be better than their previous garments with a seam. Regarding functionality, patients were significantly less limited in their daily activities when wearing JOBST Elvarex Plus garments. Finally, when wearing JOBST Elvarex Plus gloves and toe caps, patients rated the negative effect of their lymphoedema on their QoL to be significantly lower. In conclusion, the application of anatomically adapted compression garments without a seam led to improving patient's reported lymphoedema symptoms, increased patient's satisfaction with the compression garment and supported a better QoL.