Lipoedema – is a paradigm shift needed?
Last years, lipoedema has become a highly discussed topic worldwide. In 2018 and 2019, a group of lympology experts from seven European countries (members of the International Lipoedema Forum) discussed a consensus regarding (myths about) the pathophysiology of lipoedema. This resulted in the publication of the International Lipoedema Consensus Document in 2020[1], which has been presented at the 10th International Lymphoedema Framework Conference in Copenhagen, Denmark last month. The rationale of this consensus guideline has been wisely stated by (Emeritus) Professor Hugo Partsch in the foreword of the guideline:
“The time has come to establish the diagnosis of lipoedema by eliminating old, never proven concepts from the field”
But first of all… What is lipoedema?
Lipoedema is a chronic condition in women characterized by a disproportionate increase in adipose tissue in the legs (sometimes in the arms) together with pain (or/and tender to touch the skin) in this adipose tissue. This disproportional increase of adipose tissue in the legs usually starts in phases in life that are connected to hormonal changes, such as puberty, pregnancy and menopause. Research on lipoedema is scarce, and clear diagnostic tools are lacking. This contributes to the fact that the prevalence rate of lipoedema is largely unknown[1]. In a recently published US lipoedema guideline[2], it was stated that prevalence estimates range from 6.5% in children in the US[3], 6%–8% in women in Germany[4], and 15%–19%[5] in vascular clinics.
What’s in a name?
As the name implies, lipoedema is oftentimes confused with lymphedema. Also, there is a substantial variability in how clinicians approach lipoedema, its pathophysiology and treatment goals in different countries. However, the idea that lipoedema is an “oedematous” condition seems to be a lymphological dogma, according to the guideline’s authors. This perception of lipoedema results from the name given to this condition by Allen and Hines back in 1940, when the condition was first described[6]. By definition, oedema is an accumulation of fluid that manifests the classic pitting appearance of the soft tissues on clinical examination.[1] However, these first authors did not find any relevant fluid accumulation in their patients with lipoedema who were examined using a high-resolution ultrasound scan (with a 18.6MH transducer and a Moisture Meter to measure the amount of moisture). In addition, the findings on clinical inspection and ultrasound images appear to be identical in patients with lipoedema and those with pure lipohypertrophy (which is a painless disproportionate increase in adipose tissue)[1].
Consequently, purely lipoedema does not include oedema, neither is there any scientific evidence for lymphatic insufficiency.[1]** Therefore, decongestive lymphatic treatment is inappropriate for patients with purely lipoedema. Manual lymph drainage, for example, is being addressed as standard treatment for lipoedema in some international publications[7-9]. This recommendation is based on the belief that oedema lies at the root of the pain experienced in lipoedema. Nevertheless, also here the rationale for this manual lymph drainage can be questioned if no relevant oedema can be demonstrated in lipoedema, as stated by the International Consensus Guideline’s authors.
But then, if oedema is not causing the pain in lipoedema patients, what else is responsible for this painful experience in the adipose tissue? The underlying pathology of this symptom is still subject to speculation and several ideas about the cause of this pain are being addressed in literature. There is, however, a consensus that the pain is located in the subcutaneous adipose tissue of the limbs[1]. Up to now, a chronic low-grade state of inflammation and tissue hypoxia are the most plausible explanations for the pain experienced in lipoedema patients[1].
Furthermore, the experience of pain is a multifactorial phenomenon involving not only sensory but also affective, cognitive, behavioral and motivational aspects. Therefore, therapeutic approaches to lipoedema should focus on these causes of pain[1].
A paradigm shift
Based the aforementioned arguments, the authors of the consensus guideline plead for a change of the term ‘lipoedema’. Indications of both hyperplasia and hypertrophy of the adipose tissue have been found in patients with lipoedema[1]. Therefore, the deliberately non-specific term ‘lipalgia syndrome’ (‘lipos’ = fat and ‘algos’ = pain in ancient Greek) appears the most appropriate jargon, as suggested by the guideline’s authors. Redesignating ‘lipoedema’ as ‘lipalgia syndrome’ might shift the focus of the illness, which previously was the presence of oedema and its drainage although it could not be detected on clinical examination or through imaging studies[1]. In addition, as the new term focuses on the soft-tissue pain that is actually experienced by patients, rather than oedema, it also moves the therapeutic focus from oedema treatment to pain management, which has obvious benefits for patients[1].
It is hoped that the new suggested term ‘lipalgia syndrome’ will quickly become internationally recognized and gain widespread usage. So…. To be continued!
Triggered to read more, or still looking for a useful activity to spend your time on, on a dark winter evening? In the International Consensus Guideline a number of other lipoedema-related myths are being tackled as well, such as the presumptions that weight loss has no effect on lipoedema, that liposuction is effective for all lipoedema patients (and produces long-lasting effects), or that lipoedema always leads to weight gain.
Lastly, for your information:
This year an International Lipoedema Association (ILA) has been founded by 60 renowned healthcare professionals from 22 countries worldwide in an attempt to create the best possible treatment for lipoedema patients by collaborating in an international and multidisciplinary team approach based on clinical evidence and long-standing experience. Take a look at their website for more information, or for easy accessing and downloading the International Lipoedema Consensus Guideline.
Go for it!
Tessa De Vrieze
**Note: this statement has been refuted in a recent online webinar (December 7th 2021) of the LE&RN Symposium Series (which can be found here) in which Dr. Steven Dean gave a presentation about the pathomechanisms of lipo-lymphedema. In this presentation he rebutted the statement that there is no evidence available in literature by highlighting some recent papers in which a possible link between purely lipoedema patients and lymphoscintigraphic involvement has been displayed.
- Forner-Cordero et al., 2018[10]: 83 patients with lipoedema; 47% showed abnormal imaging. Abnormalities were detected in all clinical stages of lipoedema, and there was no significant association between the degree of lymphoscintigraphic involvement ant BMI (p=0.832) and the clinical stage of lipoedema (p=0.142)
- Tartaglione et al., 2020[11]: 54 patients with lipoedema (predominantly pure lipoedema and a mean BMI of 32); 75% showed abnormal imaging with characteristic tortuous lymphatics
[Note from the author: a clear number of patients with pure lipoedema, or an overview of the stages of lipoedema with the amount of patients per stage, was not provided in the paper. Therefore, results should be interpreted with caution]
References
1. Bertsch T, Erbacher G, Elwell R. Lipoedema: a paradigm shift and consensus. J Wound Care. 2020;29(Sup11b):1-51.
2. Herbst KL, Kahn LA, Iker E, Ehrlich C, Wright T, McHutchison L, et al. Standard of care for lipedema in the United States. Phlebology. 2021;36(10):779-96.
3. Schook CC, Mulliken JB, Fishman SJ, Alomari AI, Grant FD, Greene AK. Differential diagnosis of lower extremity enlargement in pediatric patients referred with a diagnosis of lymphedema. Plast Reconstr Surg. 2011;127(4):1571-81.
4. Reich-Schupke S, Schmeller W, Brauer WJ, Cornely ME, Faerber G, Ludwig M, et al. S1 guidelines: Lipedema. J Dtsch Dermatol Ges. 2017;15(7):758-67.
5. Forner-Cordero I, Szolnoky G, Forner-Cordero A, Kemény L. Lipedema: an overview of its clinical manifestations, diagnosis and treatment of the disproportional fatty deposition syndrome - systematic review. Clin Obes. 2012;2(3-4):86-95.
6. Allen EV HE. Lipedema of the legs: a syndrome characterized by fat legs and orthostatic edema. Proc Staff Mayo Clin. 1940;15:184-7.
7. Mortimer PS, Levick JR. Chronic peripheral oedema: the critical role of the lymphatic system. Clin Med (Lond). 2004;4(5):448-53.
8. Guyton AC. Pressure-volume relationships in the interstitial spaces. Invest Ophthalmol. 1965;4(6):1075-84.
9. Rutkowski JM, Stern JH, Scherer PE. The cell biology of fat expansion. J Cell Biol. 2015;208(5):501-12.
10. Forner-Cordero I, Oliván-Sasot P, Ruiz-Llorca C, Muñoz-Langa J. Lymphoscintigraphic findings in patients with lipedema. Rev Esp Med Nucl Imagen Mol (Engl Ed). 2018;37(6):341-8.
11. Tartaglione G, Visconti G, Bartoletti R, Ieria FP, Salgarello M. Rest/stress intradermal lymphoscintigraphy in diagnosis of lipedema. World J Nucl Med. 2020;19(4):376-81.