WUK BPG
       Best Practice Guidelines
       The management of lipoedema
        2017
                      Diagnosis and assessment
                      Lipoedema management
                      Life style support and  
                      self care
                      Compression therapy
                      Non-surgical and surgical 
                      interventions
                BEST PRACTICE GUIDELINES:                             EXPERT WORKING GROUP:
                THE MANAGEMENT OF                                     Tanya Coppel, Specialist Lymphoedema Physiotherapist, 
                LIPOEDEMA                                             Belfast Health & Social Care Trust, Belfast
                PUBLISHED BY:                                         Julie Cunneen, Macmillan Clinical Lead for 
                Wounds UK                                             Lymphoedema Service/Nurse Consultant, Moseley Hall 
                A division of Omniamed,                               Hospital, Birmingham
                1.01 Cargo Works                                      Sharie Fetzer, Chair, Lipoedema UK, London
                1–2 Hatfields, London SE1 9PG, UK 
                Tel: +44 (0)203735 8244                               Kristiana Gordon, Consultant in Dermatology and 
                Web: www.wounds-uk.com                                Lymphovascular Medicine, St George’s Hospital, London
                                                                      Denise Hardy, Lymphoedema/Lipoedema 
                                                                      Nurse Consultant, Kendal Lymphology Centre, 
                                                                      Kendal, Cumbria; Nurse Adviser, Lipoedema UK/
                                                                      Lymphoedema Support Network (LSN), Cumbria; Co-
                © Wounds UK, March 2017                               Chair of the Expert Working Group
                This document has been developed                      Kris Jones, Patient; Joint Managing Director & Nurse 
                by Wounds UK and is supported                         Consultant, LymphCare UK; Nurse Consultant, 
                byActiva Healthcare, BSN                              Lipoedema UK
                Medical, Haddenham Healthcare, 
                Lipoedema UK, medi UK, Sigvaris                       Angela McCarroll, Trustee, Talk Lipoedema; Patient, 
                and Talk Lipoedema.                                   Northern Ireland
                                                                      Caitriona O’Neill, Lymphoedema Care Lead Nurse, 
                                                                      Accelerate CIC, London
                                                                      Sara Smith, Senior Lecturer in Dietetics and Nutrition, 
                                                                      Queen Margaret University, Edinburgh
                                                                      Cheryl White, Lymphoedema Specialist Physiotherapist, 
                                                                      Cheshire
                                                                      Anne Williams, Lymphoedema/Lipoedema Nurse 
                                                                      Consultant, Lecturer in Nursing, Queen Margaret 
                                                                      University, Edinburgh; Trustee, Talk Lipoedema, 
                This publication was coordinated                      Edinburgh; Co-Chair of the Expert Working Group
                by Wounds UK with the Expert                          REVIEW PANEL:
                Working Group. The views                              Rebecca Elwell, Macmillan Lymphoedema CNS, Univer-
                presented in this document are                        sity Hospitals of North Midlands NHS Trust, Staffordshire
                the work of the authors and do not 
                necessarily reflect the views of the                   Peter Mortimer, Professor of Dermatological Medicine, 
                supporting companies.                                 Consultant Dermatologist, St George’s University of 
                How to cite this document:                            London
                Wounds UK. Best Practice                              Alex Munnoch, Consultant Plastic Surgeon and Clinical 
                Guidelines: The Management of                         Lead, Ninewells Hospital, Dundee
                Lipoedema.  
                London: Wounds UK, 2017.                              Dirk Pilat, General Practitioner; Medical Director for 
                Available to download from:                           ELearning at the Royal College of General Practitioners 
                www.wounds-uk.com                                     (RCGP), London
                                                                      Melanie Thomas MBE, National Clinical Lead for 
                                                                      Lymphoedema, NHS Wales and the  
                                                                      Lymphoedema Network Wales
                                                                                                                  INTRODUCTION
           Developing best practice guidelines for the 
           management of lipoedema
           People with lipoedema in the UK face              The meeting participants recognised a              GUIDE TO USING THIS 
           significant challenges. Many are not               general paucity of clinical evidence relating      DOCUMENT
           recognised by healthcare professionals as         to the management of lipoedema. The                Each section of the 
           having the condition or are misdiagnosed.         conclusions of the meeting formed the basis        document helps 
           Awareness of lipoedema among medical              for this document, which draws, where              healthcare practitioners 
           practitioners is poor, and little clinical        possible, on relevant literature. Where            to provide appropriate 
           research is focused on the condition. To          evidence is lacking, expert opinion has been       support and effective 
           date, no good quality guidelines for the          used to inform the guidelines and make             treatment and care for 
           management of the disease have been               recommendations. The content was subject           patients with lipoedema. 
           published, resulting in inconsistent and          to review by the Expert Working Group and          The key points for each 
           frequently inappropriate care for people          additional reviewers before being finalised.        section summarise 
           with lipoedema.                                                                                      the information most 
                                                             This document will be of interest to anyone        relevant to clinical 
           Even when lipoedema is diagnosed correctly,       involved in delivering support and clinical        practice
           accessing appropriate care within the NHS         services to people with lipoedema, including 
           may be difficult because of poor                    general practitioners, lymphoedema 
           understanding of treatment and referral           therapists, community nurses, plastic 
           routes, and geographical variations in clinic     surgeons, dietitians, commissioners, 
           availability, funding and capacity.               third-sector organisations and more.
           Lipoedema is a chronic, incurable disease         There is still a considerable amount to learn 
           that can have a severe impact on quality of       about lipoedema. Undoubtedly, the next few 
           life, and physical and psychosocial               years will bring rapid advances in 
           wellbeing. Some patients are so seriously         understanding of the pathophysiology of 
           affected that they lead very restricted lives,     lipoedema and the most effective ways of 
           sometimes to the extent of being unable to        managing the condition. As a result, the 
           leave their homes. The complexity of the          Group recognises that this document is likely 
           issues faced by patients with lipoedema           to need to be reviewed within three years.
           necessitates interprofessional, 
           multidisciplinary care with an emphasis on        The Group hopes that the document will be 
           supporting self management and working in         useful to people with lipoedema, and the 
           partnership with the person to identify           wide range of professionals who have 
           realistic goals and to manage expectations.       contact with them. This document is an 
                                                             early step towards achieving tangible 
           These best practice guidelines on lipoedema       benefits for patients, enhancing recognition 
           were inspired by a group of clinicians who        and diagnosis of the condition by 
           first started discussing the need for clear        professionals and the public, improving 
           guidance in 2015. The discussions                 access to best practice management, and 
           culminated in a meeting in September 2016         providing scope for future development of 
           that had the specific aim of developing            lipoedema services in the UK.
           guidelines on management that improve the 
           lives and outcomes of people with                 Anne Williams and 
           lipoedema. The meeting was ground                 Denise Hardy 
           breaking: not only did it bring together key      Co-Chairs
           opinion leaders and experts involved in the 
           treatment of lipoedema from all around the 
           UK, but, significantly, it also included people 
           with lipoedema representing UK third 
           sector organisations. 
                                                                           BEST PRACTICE GUIDELINES: THE MANAGEMENT OF LIPOEDEMA 3
            EPIDEMIOLOGY AND  
             PATHOPHYSIOLOGY 
                OF LIPOEDEMA
            SECTION 1: EPIDEMIOLOGY AND 
            PATHOPHYSIOLOGY OF LIPOEDEMA
            Lipoedema was first described in 1940 and          suggests: cases may be ‘hidden’ because of           Box 1. Synonyms for 
            is a chronic incurable condition involving a      their mild nature or because the person is           lipoedema (Schmeller & 
            pathological build-up of adipose tissue           reluctant to contact health services. Other          Meier-Vollraith, 2007; 
            (Allen & Hines, 1940). It typically affects the    cases may be unrecognised or misdiagnosed            Langendoen et al, 2009; 
            thighs, buttocks and lower legs, and              by health services. Common misdiagnoses              Herbst 2012a; Cornely, 
            sometimes the arms, and may, although not         include obesity or lymphoedema (Box 2)               2014)
            always, cause considerable tissue                 (Goodliffe et al, 2013), although both                ■  Adiposalgia
            enlargement, swelling and pain. It may            conditions may co-exist with lipoedema.              ■  Adiopoalgesia
            significantly impair mobility, ability to                                                               ■  Lipalgia
            perform activities of daily living, and           Cause                                                ■  Lipedema (American 
            psychosocial wellbeing. Current                   The precise mechanisms responsible for the              spelling)
            conservative management involves                  development of lipoedema are unknown,                ■  Lipohyperplasia dolorosa
            encouraging self-care, managing symptoms,         but it is likely that multiple factors are           ■  Lipohypertrophy 
            improving functioning and mobility,               involved (Okhovat & Alavi, 2014).                       dolorosa 
            providing psychosocial support, and                                                                    ■  Lipomatosis dolorosa of 
            preventing deterioration in physical and          Lipoedema often first presents during                    the legs
            mental health and wellbeing.                      puberty, although oral contraceptive use,            ■  Painful column legs
                                                              pregnancy and the menopause also appear              ■  Painful fat syndrome
            Lipoedema is predominantly a chronic              to be triggers. These observations suggest           ■  Riding breeches 
            adipose tissue disorder (the word lipoedema       that hormonal change may be involved                    syndrome
            means ‘fat swelling’), with clinically apparent   in initiating the characteristic build-up of         ■  Stovepipe legs.
            oedema due to fluid accumulation in the            adipose tissue (Fonder et al, 2007; Bano et 
            tissues occurring as a secondary feature in       al, 2010; Godoy et al, 2012). Onset of the 
            some individuals (Todd, 2010; Herbst,             disease after periods of significant weight          Box 2. Lymphoedema and 
            2012a; Reich-Schupke et al, 2013; Herbst et       gain have also been reported (personal              lipoedema (Harwood et 
            al, 2015). Although most commonly called          communication, K Gordon).                           al, 1996; Lymphoedema 
            lipoedema, the condition has a variety of                                                             Framework, 2006;  
            other names (Box 1).                              There is also evidence of a genetic                 Goodliffe et al, 2013)
                                                              predisposition to lipoedema. A family                Patients with lipoedema 
            Prevalence                                        history of the condition has been found              may be misdiagnosed as 
            Lipoedema almost exclusively affects               in 15%–64% of patients (Harwood et                   having lymphoedema. 
            women, but a few cases have been reported         al, 1996; Child et al, 2010; Schmeller &             Lymphoedema results 
            in men (Chen et al, 2004; Langendoen et al,       Meier-Vollrath, 2007). The genetic variants          from malfunction of the 
            2009). Relatively little epidemiological          involved have not been identified fully,              lymphatic system, whereas 
            research has been carried out on lipoedema        but research suggests that autosomal                 lipoedema is thought to 
            and so it is unclear exactly how many             dominance with male sparing is the most              primarily be a disorder 
            people are affected and to what extent.            likely mode of inheritance (Child et al,             of adipose tissue (a 
            The research so far has produced widely           2010). Investigations into the genetics              lipodystrophy). Confusingly, 
            varying figures. In the UK, the minimum            of lipoedema are ongoing, and include                however, patients with 
            prevalence of lipoedema has been estimated        researching whether men may act as                   lipoedema may develop 
            to be 1 in 72,000 (Child et al, 2010).            carriers for the associated genetic factor(s).       lymphatic dysfunction. 
            However, the authors noted that this is                                                                This combination of 
            likely to be an underestimate (Child et al,                                                            lipoedema and secondary 
            2010). In Germany, the prevalence of                                                                   lymphoedema is 
            lipoedema has been estimated to be 11% in                                                              sometimes referred to  as 
            women and post-pubertal girls (Földi et al,                                                            lipolymphoedema.
            2006; Szél et al, 2014).
            Further research is needed to establish 
            clearly the proportion of the population 
            affected by lipoedema. It is likely to be more 
            common than the limited evidence available 
           4    BEST PRACTICE GUIDELINES: THE MANAGEMENT OF LIPOEDEMA