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Lipedema: a misdiagnosed and misunderstood fatty deposition syndrome
1.
2. Lipedema
symmetrical edema in the lower limbs with fatty
deposits located on hips and thighs
chronic disease that occurs mostly in females
hypothesis: estrogen regulated polygenetic disease
leading to vascular and lymphatic abnormalities
causing inflammation affecting peripheral nerves
main disorders considered for differential diagnosis:
lymphedema, primary or secondary
obesity
lipohypertrophy
venous edema
3. Disclosures
I have no actual or potential conflict of
interest in relation to this
program/presentation
4. Lipedema stages
Fatty accumulation on hips, thighs, legs later torso
and arms; feet swelling on later stages (III -IV), upper
arm swelling on later stages (III- IV)
Stage I – “thick legs”, subtle skin indentations on
upright position, soft skin, small fatty nodules
Stage II – more pronounced skin indentations, fatty
nodules palpable of different sizes, loss of skin
elasticity, superficial hematomas may be present
5. Lipedema stages
Stage III – pronounced skin stretching,
prominent hanging fat pads and masses
mostly inner thighs and knees, and
upper arms, hardening of the tissues,
vascular fragility
Stage IV – large fat masses
and hanging lobules on legs and
arms, varicosities may be present,
variations of skin changes including
trophic, hardening of the skin and
tissues, joint deformities may be
present
6. Chief complaints
pain mostly on legs
easy bruising
leg and arm swelling
fatty lumps on legs, abdomen and arms
heaviness
weakness
fatigue, tiredness
swelling and fatty deposition worsening after puberty,
pregnancies, contraceptive pills
8. Physical Examination
symmetric fatty deposition on legs, abdomen, arms
superficial varicosities may be present
tenderness to palpation most prominent at pretibial area
bruises, varicosities
hypermobile joints
non-pitting edema, usually feet
are spared
negative Stemmer sign (thickened
skin at base of 2nd toe or 2nd finger),
positive Stemmer sign in
lipo-lymphedema
10. Staging I-IV and BMI
47.37 47.29
41.66 40.43 37.20
33.65 32.03 31.78
24.82
57.51
44.11
47.42
0.00
10.00
20.00
30.00
40.00
50.00
60.00
70.00
I II II II II II II II II III III IV
BMI
Stages
11. Comorbidities
diabetes mellitus type II, glucose intolerance
high blood pressure
obesity
arthritis mostly osteoarthritis at spine, hips and knees
thyroid disease; hypothyroidism, goiter, Hashimoto’s
thyroiditis
fibromyalgia syndrome
15. Surgical history
gastric sleeve
gastric bypass
removal of excess skin after bariatric surgery –
abdomen, thigh lift, arms
hysterectomy
cholecystectomy
total knee replacement
lumbar laminectomy and fusion
16. Laboratory abnormalities
Low vitamin D - usually <15 ng/mL (deficiency)
Elevated CH50 – presence of chronic inflammation
Elevated homocysteine - an amino acid and
breakdown product of protein metabolism when
present in high concentrations has been linked to an
increased risk of heart attacks and strokes
Elevated cholesterol and/or triglycerides
Low HDL, high LDL
Elevated fasting blood glucose, high A1C
Low IgG, IgA – immunodeficiency
17. Laboratory abnormalities
Elevated IgE - allergies
Elevated liver enzymes – fatty liver
Elevated factor VIII clotting activity – risk factor for
deep venous thrombosis (blood clots)
Elevated creatinine clearance and low eGFR – kidney
insufficiency
Low hemoglobin level, iron deficiency (low total iron
and ferritin) - anemia
Low cortisol a.m. level – adrenal insufficiency
18. Lymphoscintigraphy
intradermal or subcutaneous radiotracer injection
between toes, fingers
evaluates the lymph flow, obstruction and
abnormalities of the lymphatic systems
19. Lymphoscintigraphy
estimates the uptake of a radiolabeled
tracer (Tc-99m Sulfur colloid) that is
transported into the regional lymph
nodes by the lymphatic system
routinely performed as part of evaluation of a swollen
limb
this technique might both determine the underlying
cause of swelling and indicate its pathophysiology
24. Echocardiogram
Left ventricular hypertrophy
Mild diastolic dysfunction
Trace, mild aortic and mitral valve regurgitation
Dilated left atrium
67% (n=6)
25. Treatment
Manual lymphatic drainage (MLD) / decongestive
therapy / application of multilayer compression
bandages (foam, short stretch bandages)
Use of compression garments; stockings, arm sleeves,
Capri 20 – 30 mmHg; micro massaging garments -
better tolerated
Night garments and inelastic compression garments –
poorly tolerated
Get proper sleep and exercise
26. Exercise
Low impact aerobic exercises
Underwater exercises
General stretching
Strengthening – progressive
resistance – elastic bands,
tubing
Studies concluded that slowly progressive exercise of varying
modalities is not associated with the development or
exacerbation of lymphedema such as aerobic and strengthening
In patients with persistent systemic inflammation as seen in
lipedema and lymphedema, regular exercise training lowers
levels of pro-inflammatory cytokines
27. Chronic Lymphedema
Rockson, S. G. (2013). The Lymphatics and the Inflammatory Response: Lessons
Learned from Human Lymphedema. Lymphatic Research and Biology, 11(3), 117-120.
29. Interventions
Corrections of vitamin deficiencies such as vitamin D,
B12, folate, B6
Reduction of homocysteine levels with high doses of
vitamin B12, B6 and folate
Replace minerals i.e. zinc, magnesium
Antioxidants & anti-inflammation – curcumin,
green tea, resveratrol, vitamin C
30. Selenium
antioxidant properties, trigger immune activation
Good source: 1 medium brazilian nut = 95 mcg, 2 nuts
a day.
Dose: 200 mcg once a day
31. Bioflavonoids
essential for the proper absorption
and utilization of vitamin C
increase the strength of the
capillaries, and help to prevent
hemorrhages and ruptures, while
also building a protective barrier
against infection
citrus bioflavonoids, diosmin
Dose: 500mg once to twice a day
33. Beta 1,3/1,6 glucans
powerful antioxidant, moderate tissue-damaging
cytokines
activation of macrophages, neutrophils, and T-cell–
mediated immunity
assist in ameliorating microbial imbalance – fight
infections
Foods: cereal β-glucans from oat, barley, wheat and rye;
mushroom β-glucans from shiitake mushrooms
Dose: 500mg / day on empty stomach with 8 ounce
water
34. Liposuction
Water-jet assisted (WAL) and tumescent liposuction
36 y/o female patient underwent WAL for stage I
lipedema with good results, later had a thigh lift for
loose skin. Lymphoscintigraphy showed slightly
prominent lymphatic channels in the ankle joint and
distal calves bilaterally right > left, inguinal lymph
nodes were identified at 11 minutes; and iliac nodes
at the iliac chains visualized bilaterally @ 30
minutes. One year after WAL + diet + exercises
lipedema remained under control.
35. Conclusion
Lymphatic abnormalities are evident in early stages
Wide array of comorbidities are present
Multiple treatments and interventions – effectiveness
and long-term outcomes are unknown
36. References
Blome, C., Augustin, M., Heyer, K., Knöfel, J., Cornelsen, H., Purwins, S., & Herberger, K. (2014). Evaluation of Patient-
relevant Outcomes of Lymphedema and Lipedema Treatment: Development and Validation of a New Benefit Tool.
European Journal of Vascular and Endovascular Surgery, 47(1), 100-107.
Boursier V., Pecking A., Vignes S. (2004). Comparative analysis of lymphoscintigraphy between lipedema and lower
limb lymphedema. J Mal Vasc, 29(5), 257-61.
Cuzzone, D. A., Weitman, E. S., Albano, N. J., Ghanta, S., Savetsky, I. L., Gardenier, J. C., . . . Mehrara, B. J. (2014). IL-6
regulates adipose deposition and homeostasis in lymphedema. AJP: Heart and Circulatory Physiology, 306(10).
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manifestations, diagnosis and treatment of the disproportional fatty deposition syndrome - systematic review. Clinical
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Lymphatic Research and Biology, 11(3), 117-120.
37. References
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12(3), 123-123.
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