Lipedema Overview
Lipedema, or painful fat syndrome, is characterized by excess fat developing below the waist and extending down to the ankles but stopping above the feet. About 80% of cases involve both legs and arms; only 3% are arms only. The fat is symmetric (in the early stages), tender, often painful, and bruises easily.
Types of lipedema are defined based on the affected areas (see sidebar Types and Photos of Lymphedema and Lipedema). Lipedema develop along with lymphedema, obesity, or other less common adipose tissue disorders, such as Dercum’s disease or multiple symmetric lipomatosis.
Dercum’s disease (adiposis dolorosa) is characterized by very painful nodular subcutaneous adipose tissue (SAT) and other signs and symptoms that typically develop around the age of 35 (although Dercum’s can occur in children and older individuals). Clinically people with Dercum’s disease can look like they have lipedema or familial multiple lipomatosis. The pain is chronic, can move around, is often disabling, and resistant to traditional analgesics. Associated symptoms include easy bruising, gastrointestinal complaints, joint and muscle pain, sleep disturbances, impaired memory, depression, difficulty concentrating, anxiety, rapid heartbeat, shortness of breath, diabetes, bloating, constipation, fatigue, weakness and joint aches.
Multiple symmetric lipomatosis (MSL) is a rare syndrome characterized by the painless, symmetrical accumulation of abnormal tumor-like SAT, either as discrete non-encapsulated lipomas or as a flowing increase in SAT. The appearance and location of SAT in MSL can vary. Individuals with MSL have increased SAT in a symmetrical distribution on the neck, the back, the chest, the upper arms, or on the thighs. MSL usually spares the extremities but altered fat may involve the whole body. Fat and muscle wasting can occur in other areas [i].
Characteristics that distinguish lipedema from lymphedema include:
Pain: lipedema patients often complain of pain when touched; particularly on the legs. For example, when their cat walks on them.
Bruising and subcutaneous bleeding may occur from impact or spontaneously.
Tissues affected by lipedema alone are not prone to the infections that characterize lymphedema. Infections are more likely after lymphedema swelling develops.
In the early stages of lipedema, the upper part of the body may remain slim while fat accumulates from the tops of the hips to the ankles without involving the feet [[ii]]. The feet are spared, swelling stops at the ankles, and a skin-fold test (Stemmer sign) on the top of the foot or toes is negative.
Weight gain accumulates evenly on the affected areas on both legs from the top of the hip down to the ankle. Leg fat from lipedema appears more symmetric than swelling from lymphedema.
Weight loss comes only from areas that are not affected by lipedema. There may be weight loss from the legs if lipedema is combined with obesity.
Swelling in the legs usually gets worse after standing, during times of increased heat, and during the second half of the day. Some individuals experience reduced swelling during sleep; others do not experience this benefit.
Lipedema skin is smooth initially but over time, fatty lumps called nodules develop within the affected tissues and the skin appears lumpy.
In the later stages of lipedema, larger, rounded, fat deposits called lobules develop making legs irregularly shaped and interfering with posture and walking.
Lipedema progresses through these stages:
- Stage 1: Normal skin surface with enlarged areas of fat.
- Stage 2: Uneven skin with indentations in the fat (cellulite), the surface of the skin takes on the lumpy appearance of a mattress with fat globules bulging between thickened connective fibers (fibrotic septa).
- Stage 3: Large extrusions of tissue causing deformations especially on the thighs and around the knees.
- Stage 4: Lipedema with lymphedema (lipolymphedema); abnormal fat on the hands, feet, trunk and head.
Lipedema is rarely seen in Asian women. Lipedema in men is very rare and may be linked to abnormal hormone levels or liver disease.
Lipedema typically appears during puberty and increases during pregnancy or menopause. Changes in body shape from lipedema can start before puberty and lipedema fat on legs and arms may be apparent by the age of eight. One pediatric clinic reported that 6.5% of patients referred for lymphedema actually had lipedema [[iv]].
In very early stage lipedema, the skin in affected areas looks normal and the fat is very smooth and soft. Over time, the fat becomes increasingly nodular. When lipedema progresses to more advanced stages, the rectangular compartments of fat and connective tissue below the skin become larger and protrude, causing a mattress-like appearance that can include larger fatty lumps (lipomas). Fat on the inner legs can form lobules that restrict mobility and damage the knees and other joints.
Lipedema fat alone does not harm the heart or metabolism as does abdominal fat. Women can have large amounts of lipedema fat and be considered obese, yet remain metabolically healthy if they avoid abdominal obesity.
Although lipedema is an inherited condition, the specific genes involved have not been identified. Inheritance appears to be autosomal dominant, passing from mother or father to daughter.
Lipedema causes visible lymphedema swelling in the later stages and swelling may extend into the feet. The combined condition is lipolymphedema [[v]]. Lymphedema care recommendations apply to both conditions.
One characteristic of lipedema is persistent enlargement of the hips, buttocks and legs. Fat in these areas remains unaffected by weight loss efforts involving diet, exercise, weight loss medications, or weight loss surgery, even though weight is lost from unaffected areas, usually the trunk.
Types of Lipedema
European Lymphology Society sub-classification system for types of lipedema based on segments of the lower body affected allows for a mixture of types in a given individual:
- Type I: buttock and hips (saddle bag phenomena).
- Type II: buttock to knees.
- Type III: buttock to ankles.
- Type IV: arms and legs affected.
- Type V: lipolymphedema.
UK Guidelines use different types:
- Type I affects the lower abdomen down over the hips and buttocks.
- Type II affects lower abdomen down to the knees.
- Type III affects the lower abdomen to the ankles.
- Type IV is when the arms are affected.
- Type V affects primarily the lower leg
Most women have Types II and IV or Types III and IV.
Coppel T, Cunneen J, Fetzer S, et al. Best Practice Guidelines: The management of lipoedema. Wounds UK. 2017;13(1).
Portions adapted from Lymphedema and Lipedema Nutrition Guide, Lymph Notes 2016 by permission of the publisher.
[i] Herbst, KL. Rare Adipose disorders (RAD) masquerading as obesity. Acta Pharmalogica Sina 2012 33:155-172. www.ncbi.nlm.nih.gov/pubmed/22301856
[ii] MacDonald, J.M. et al “Lymphedema, Lipedema, and the Open Wound: the role of compression therapy.” Surg Clin North Am. 2003 Jun:83(3):639-58. www.ncbi.nlm.nih.gov/pubmed/12822730
[iii] Hodson S, Eaton, S. Lipoedema management: gaps in our knowledge. Journal of Lymphoedema, 2013, Vol 8, No 1. Available from www.woundsinternational.com/media/issues/921/files/content_11243.pdf
[iv] Schook CC, et al. Differential diagnosis of lower extremity enlargement in pediatric patients referred with a diagnosis of lymphedema. Plast Reconstr Surg. 2011 Apr;127(4):1571-81. www.ncbi.nlm.nih.gov/pubmed/21187804
[v] Herbst, KL. Rare Adipose disorders (RAD) masquerading as obesity. Acta Pharmalogica Sina 2012 33:155-172. www.ncbi.nlm.nih.gov/pubmed/22301856