BMN Blog

APR 02

Lipedema (Lip- fat, edema- swelling) is a disease of abnormal and disproportionate adipose tissue deposition almost exclusively occurring in women1. While the disorder was originally described in 19402, lipedema remains under-recognized and underdiagnosed in the United States3. This article aims to elucidate the salient features of lipedema toward the goal of raising awareness among the medical community.

 

As mentioned above, lipedema occurs nearly exclusively in women with the exception of a few case reports4. In addition, a prevalence of up to 11% of the female population has been estimated1. In view of the high estimated prevalence, and in combination with the serious clinical course outlined below, it behooves us as physicians to recognize, diagnose, and refer patients with this disease appropriately. At the same time, given the absent or minimal exposure to lipedema in medical education, our lack of familiarity of this disorder is entirely understandable.

 

The defining features of lipedema are symmetrical and disproportionate deposition of subcutaneous adipose tissue in the lower extremities, pain and tenderness in the affected areas, along with dependent edema and vascular fragility4. While there are several identified distributions of associated lower extremity adiposis, the proximal legs (buttocks and hips) are nearly always involved (Figure 1), and regardless of the distal extent of involvement, lipedema never involves the feet except in the most advanced cases when lymphedema has developed5. As a result, an “ankle cuff” (Figure 2) is frequently seen at the junction of affected and unaffected tissue6. Physical examination will also typically reveal a granular or nodular texture of the enlarged subcutis that has been likened to “a bag of round peas”7 or “grains of sand”8. In addition, palpation of the lower extremities will elicit tenderness9. Furthermore, the overlying skin will show evidence of capillary fragility in the form of petchiae, telangiectasias, or ecchymosis. Patients will also give a history of easy bruisability3.

 

With respect to the patient’s history, an almost universal finding will be extensive efforts at weight loss (often including bariatric surgery), with varying efficacy in the upper body but little to no results in the areas affected by lipedema6. This point is a source of substantial frustration for affected patients, especially since they are frequently diagnosed with simple obesity and advised by medical professionals to lose weight8. Oftentimes these individuals will exhibit moderate to severe functional limitations including decreased mobility, altered gait, and accelerated degeneration of the hips and knees10.

 

Lipedema typically begins and worsens coincident with periods of hormonal changes such as puberty, pregnancy, and menopause1. There also appears to be a familial nature to this disease, with 16-45% of affected individuals noting such a pattern11,12.

 

Although much of the pathophysiology is as yet poorly understood, several therapeutic strategies have been proved effective. What is clear is that if left untreated, lipedema progresses in severity, symptomatology, and impact13. A multidimensional approach including comprehensive decongestive therapy, nutritional supplementation and physical activity, along with psychological and emotional support, has shown significant improvement in patients with lipedema14,15,16,17,18. From an interventional perspective, adipose removal strategies including specialized liposuction techniques have resulted in dramatic results in terms of pain reduction, functional status, and disease progression19,20,21,22,23.

 

In conclusion, lipedema is an alarmingly common, highly impactful, and effectively treatable disease which remains under-recognized among the medical community. With greater awareness, the large subset of our female population affected by this disorder can be identified and referred for appropriate management. The Alabama PVD Center is committed to educating medical professionals and the general public about lipedema and we welcome inquiries about our interests and services. Please visit our website http://alpvd.com/ for more information.

 

Figure 1: Ankle-Cuff Sign in Lipedema. Courtesy Catherine Seo, Lipedema Project. Used with permission.

Figure 1

 

Figure 2: The Four Stages of Lipedema. Courtesy Catherine Seo, Lipedema Project. Used with permission.

figure 2

 


 

References

  1. Foldi E, Foldi M, et al. Foldi’s Textbook of Lymphology, 3rd ed. Urban & Fischer, 2012.
  2. Allen EV, Hines EA. Lipedema of the legs: a syndrome characterized by fat legs and orthostatic edema. Proc Staff Meet Mayo Clin. 1940;15:184-187.
  3. Fife CE, Maus EA, Carter MJ. Lipedema: a frequently misdiagnosed and misunderstood fatty deposition syndrome. Adv Skin Wound Care. 2010;23(2),81-92.
  4. Dayan E, Kim J, et al. Lipedema - the Disease They Call Fat: An Overview for Clinicians. Lipedema Simplified Publications, 2017.
  5. Child AH, Gordon KD, Sharpe P, et al. Lipedema: an inherited condition. Am J Med Genet A. 2010;152A(4):970–976.
  6. Langendoen SI, Habbema L, Nijsten TE, Neumann HA. Lipoedema: from clinical presentation to therapy. A review of the literature. Br J Dermatol. 2009;161(5):980-986.
  7. Buck DW, Herbst KL. Lipedema: A relatively common disease with extremely common misconceptions. Plast Reconstr Surg - Glob Open. 2016;4(9):e1043.
  8. Herbst, K. Rare Adipose Disorders (RADs) masquerading as obesity. Acta Pharmacol Sin. 2012; 33(2):155-172.
  9. Shin BW, Sim J-Y, Jeong HJ, Kim GC. Lipedema, a rare disease. Ann Rehabil Med. 2011;35(6):922-927.
  10. Goodliffe JM, Ormerod JOM, Beale A, Ramcharitar S. An under-diagnosed cause of leg swelling. BMJ Case Reports. 2013. doi:10.1136/bcr-2013-009538.
  11. Harwood CA, Bull RH, Evans J, Mortimer, PS. Lymphatic and venous function in lipoedema. Br J Dermatol. 1996;134(1):1-6.
  12. Bano G, Mansour S, Brice G, et al. Pit-1 mutation and lipoedema in a family. Exp Clin Endocrinol Diabetes. 2010;118(6):377-380.
  13. Okhovat J, Alavi A. Lipedema: A review of the literature. Int J Low Extrem Wounds. 2015;14(3):262-267.
  14. Reich-Schupke S, Schmeller W, Brauer WJ, Cornely ME, Faerber G, Ludwig M, Lulay G, Miller A, Rapprich S, Richter DF, Schacht V, Schrader K, Stücker M, Ure C. S1 guidelines: Lipedema. J Dtsch Dermatol Ges. 2017;15(7):758-767.
  15. Szolnoky G, Varga E, Varge M, et al. Lymphedema treatment decreases pain intensity in lipedema. Lymphology. 2011;44(4):178-182.
  16. Szolnoky G, Borsos B, Bársony K, et al. (2008). Complete decongestive physiotherapy with and without pneumatic compression for treatment of lipedema: A pilot study. Lymphology. 2008;41(1):40-44.
  17. Keith L, Rowsemitt C, Richards LG. Lifestyle modification group for lymphedema and obesity results in significant health outcomes. American Journal of Lifestyle Medicine.
  18. Dudek JE, Białaszek W, Ostaszewski P. Quality of life in women with lipoedema: A contextual behavioral approach. Qual Life Res. 2016;25(2):401-408.
  19. Baumgartner A, Hueppe M, Schmeller W. Long‐term benefit of liposuction in patients with lipoedema: a follow‐up study after an average of 4 and 8 years. Br J Dermatol. 2016;174(5),1061-1067.
  20. Dadras M, Mallinger PJ, Corterier CC, Theodosiadi S, Ghods M. Liposuction in the treatment of lipedema: A longitudinal study. Arch Plast Surg. 2017;44(4):324-331. doi: 10.5999/aps.2017.44.4.324.
  21. Stutz JJ, Krahl D. Water jet-assisted liposuction for patients with lipoedema: Histologic and immunohistologic analysis of the aspirates of 30 lipoedema patients. Aesthetic Plast Surg. 2009;33(2):153-162.
  22. Peled A, Slavin S, Brorson H. Long-term outcome after surgical treatment of lipedema. Annals Plast Surg. 2012;68(3):303-307.
  23. Wollina U, Heinig B, Nowak A. Treatment of elderly patients with advanced lipedema: a combination of laser-assisted liposuction, medial thigh lift, and lower partial abdominoplasty. Clin Cosmet Investig Dermatol. 2014;23(7):35-42.
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