Lipedema

Lipedema is a disorder of adipose tissue distinguished by five characteristics: 1) it can be inherited; 2) it occurs almost exclusively in women; 3) it can occur in women of all sizes, from the anorexic to the morbidly obese; 3) it involves the excess deposit and expansion of fat cells in an unusual and particular pattern – bilateral, symmetrical and usually from the waist to a distinct line just above the ankles; and 4) unlike the “normal” fat of obesity, lipedemic fat cannot be lost through diet and exercise. Surgery is highly controversial, and in many cases, can make the condition worse.

Lipedema usually is triggered at puberty, but can trigger or worsen during or after pregnancy, at peri-menopause, and following gynecological surgery. If lipedema is diagnosed early, which currently is very rare, it is possible to prevent a significant expansion of lipedemic fat cells, and to alert patients to their heightened risk for obesity so they can take appropriate action.

Estimates of the incidence of lipedema vary widely, and range as high as 11% of the post-puberty female population. Even if the number were only 5%, that would mean more than 6 million women in the United States alone.

Differentiating lipedema from other diseases

Lipedema must be differentiated from other causes of lower limb edema like chronic venous insufficiency, acute deep venous thrombosis, lymphatic filariasis, myxedema, cellulitis and causes of generalized edema.

Diseases	Symptoms							Signs	Gold standard Investigation to diagnose
	History	Onset	Pain	Fever	Laterality	Scrotal swelling	Symptoms of primary disease
Lipedema	Family history especially in women; X-linked dominant or autosomal dominant condition. Abnormal deposition of fat and edema and easy bruising.	Chronic	+	-	Bilateral	-	-	Tender with palpation Negative Semmer sign to differentiate from lymphedema. Pinching the skin on the upper surface of the toes. If it is possible to grasp a thin fold of tissue then it is negative result. In a positive result, it is only possible to grasp a lump of tissue.	MRI offers strong qualitative and quantitative parameters in the diagnosis of lipedema
Lymphatic filariasis	History of living in endemic area or travelling to it	Chronic	+	+	Bilateral	+	-	Hepatomegaly Lymphedema Elephantiasis Lymphangitis Hydrocele Scrotal elephantiasis Lymphadenopathies Rhonchi may be present in patients with Pulmonary tropical eosinophilia syndrome.	Preparing blood smears Thick smears Thick smears consist of a thick layer of dehemoglobinized (lysed) red blood cells (RBCs). Thick smears allow a more efficient detection of parasites (increased sensitivity). Thin smears consist of blood spread in a layer such that the thickness decrease. By the ultrasound, the following findings can be observed: Dilated lymphatic channels Living worms tend to be in motion which called "filarial dance" sign.
Chronic venous insufficiency	History of untreated varicose veins Painful bilateral lower limb swelling that increases with standing and decreases by rest and leg elevation.	Chronic	+	-	Bilateral	+ (If congenial)	-	Typical varicose veins Skin change distribution correlate with varicose veins sites in the medial side of ankle and leg Reduction of swelling with limb elevation.	Duplex ultrasound will demonstrate typical findings of venous valvular insufficiency
Acute deep venous thrombosis	History of prolonged recumbency Classic symptoms of DVT include acute unilateral swelling, pain, and erythema	Acute	+	-	Unilateral	-	May be associated with primary disease mandates recumbency for long duration	Dilated superficial veins Difference in calf diameter is twice as likely to have DVT(most impotant sign ) Calf pain on passive dorsiflexion of the foot (Homan's sign) isn't realiable sign.	Compression ultrasonography (CUS) with doppler is the diagnostic test of choice D-dimer level is used for unprobable cases
Myxedema	History of untreated hypothyroidism. Infiltration of the skin with glycosaminoglycans with associated water retention.	Chronic	+	-	Bilateral	-	+ (hypothyroidism )	Pretibial myxedema	Thyroid function tests.
(Cellulitis-erysipelas-skin abscess)	Acute painful swelling Fever	Acute	+	+	Unilateral	-	-	Tenderness, hotness, and may be fluctuation if abscess formed. Lymphangitis in nearby lymph nodes. Toxemia and fever in severe cases. Cellulitis involves the deeper dermis and erysipelas involves the upper dermis.	Usually it doesn't need any laboratory tests to diagnose. Blood cultures are warranted for patients in the following circumstances: Systemic toxicity Extensive skin or soft tissue involvement Underlying comorbidities persistent cellulitis In patients with recurrent cellulitis, serologic testing for beta-hemolytic streptococci is a good diagnostic tool.
Other causes of generalized edema	History of chronic general condition (cardiac-liver-renal)	Chronic	-	-	Bilateral	-	+		According to the primary cause ( Echo- LFTs- RFT)

Symptoms

Patients tend to gain weight in lipedemic areas and lose it in non-lipedemic areas. Obese lipedema patients who undergo bariatric surgery lose fat primarily from the waist up. Even anorexic women can starve and exercise away "normal" fat but retain lipedemic fat.

The classic early-stage lipedemic profile is a woman who looks like a size 8 from the waist up and a size 16 from the waist down with disproportionately large, column-like legs. As lipedema progresses, patients become increasingly heavy in the lower body. The additional, expanding fat cells interfere with the pathways of lymphatic vessels, and patients can develop secondary lymphedema, a condition known as lipo-lymphedema. Many lipedema patients cannot tolerate the compression garments associated with conventional lymphedema treatment because the underlying lipedemic fat is very painful, and those patients therefore are at risk for the side effects of uncontrolled lymphedema, including recurring blood infections and fibrosis.

Women with lipedema also are at very high risk for obesity because the lipedemic fat cannot be lost, and because as the condition worsens, patients become progressively less mobile.

Treatment

Treatment is designed primarily to address the secondary lymphedema part of the lipedema patient’s condition. This treatment includes a course of manual lymphatic drainage and bandaging by a lymphedema therapist, followed by the wearing of custom-fitted compression garments or devices - usually stockings and sometimes biker shorts. Compression prevents recurrence of lymphedema, and in some lipedema patients can reduce the pain of lipedemic fat.

There is no cure for lipedema.

History

Although first identified in the United States, at the Mayo Clinic in 1940, lipedema is barely known in that country – to physicians or to the patients who have the disease. Lipedema often is confused with obesity, and a significant number of patients currently diagnosed as obese are believed to have lipedema, either instead of or in addition to obesity.

Lipedema has multiple spellings. "Lipedema" is the American spelling, while "Lipoedema" is used in Britain and Europe. "Lipodema" is also used occasionally.

References

• Interview with the Founder and President of the National Lipedema Association

• "Lipedema: What Do We Know?" by Professor Horst Weissleder, MD

• "Lipedema," on the web site of Lymphedema Therapy, Woodbury, New York

• Lipedema and Lymphedema compared

• National Lipedema Association

• A Lipedema patient's informative website

• Xanga Lipedema blog

• Big Leg Woman, a blog about lipedema

• Coyote Dreamin' a blog about life with Lipedema and Lymphedema

• Lipedema Yahoo Support Group