Developmental Disorders of the Lymphatics

An information blog for disorders of the lymphatics. For all articles, please click on "Archives" - Due to spammers, I will no longer allow comments, sorry.

Saturday, August 26, 2006



Overview and Etiology.

The term lipedema was first used by Allen and Hines (1940) to describe a symmetrical "swelling" of both legs, extending from the hips to the ankles, caused by deposits of subcutaneous adipose (fatty) tissue. The underlying etiology of these fat deposits remains unknown. While lipedema is not a disorder of the lymphatic system per se, it is frequently confused with bilateral lower extremity lymphedema. It occurs almost exclusively in women and may have an associated family history (20 per cent of cases) and is usually accompanied by hormonal disorders as well (Strossenreuther, 1999). If present in a man, it is accompanied by massive hormonal disorder. Fat in the lower extremities extends to the malleoli (ankle bones), often with flaps of tissue hanging over the foot. The feet are not affected; occasionally, lipedema is found in the arms. Typically, there are also fatty bulges in the medial proximal thigh and the medial distal thigh, just above the knee. Clinically, the affected individuals complain of pitting edema as the day progresses, which is relieved by prolonged elevation of the leg(s) overnight (Rank and Wong, 1966; Rudkin and Miller, 1994; Casley-Smith, 1997).

Stages of Lipedema.
In Stage I, the skin is still soft and regular, but nodular changes can be felt upon palpation (see photo #1). There are no color changes in the skin and the subcutaneous tissues have a spongy feel, like a soft rubber doll. In Stage II, the subcutaneous tissue becomes more nodular and tough. Large fatty lobules begin to form on the medial distal and proximal thigh and medial and lateral ankles just above the malleoli (see photo # 2). Pitting edema is common, increasing as the day progresses. The individual may report hypersensitivity over the anterior tibial (shin) area. Skin color changes occur in the lower leg, indicative of secondary lymphedema, which often occurs in later stage lipedema.

Pathophysiology of Lipedema (Strossenreuther, 1999).

There are many histological and physiological changes that occur in lipedema. There is a decrease in the elasticity of the skin and underlying connective tissue. The basement membrane of blood vessels is thickened and there are disturbances in vasomotion. There is decreased vascular resistance, increased skin perfusion, and increased capillary filtration. There is increased venous/blood capillary pressure causing increased ultrafiltration.

These vascular changes combined with the decreased efficiency of the calf muscle pump, result in both the dependent pitting edema seen in Stage I, as well as the the secondary lymphedema that often complicates lipedema in its later stages. Histological changes seen in lipedema include a thinning of the epidermal layer, thickening of the subcutaneous tissue layer, fibrosis of arterioles, tearing of elastic fibers, dilated venules and capillaries, and hypertrophy and hyperplasia of fat cells. Clinical studies show that there is enlargement of the pre-lymphatic channels (Stoberl et al., 1986) as well as defects in capillary perfusion (Weinert and Leeman, 1991). Some authors have reported no alteration in lymphatic transport (Brautigam et al., 1998) while others (Bilancini et al., 1995) have reported decreased lymph outflow in those individuals with lipedema. Foldi and Foldi (1993) reported an increase in fat cell growth during lymphostasis.
Medical ManagementDiagnosis. The diagnosis of lipedema is difficult if the clinician is unfamiliar with this condition. Often, these people are told that they are "fat" and should just lose weight to resolve the problem. For reasons still unknown, the fatty tissue accompanying this condition cannot be significantly decreased by diet. It is not uncommon for a diagnosis of primary lymphedema to be made. This results in frustration for the person who then seeks out lymphedema therapy with poor results.

There are several significant clinical differences between lipedema and bilateral primary lymphedema. The feet are not involved in lipedema; while they are edematous with a positive Stemmer's sign in lymphedema, Stemmer's sign is negative in lipedema (see fig. 12-17). The "swelling" in lipedema is symmetrical, while in primary lymphedema usually one limb is more involved than the other. The subcutaneous tissues feel rubbery in lipedema. In advanced Stage II lymphedema, there is significant subcutaneous fibrosis, which feels firmer than lipedema. While there have been reported incidences of cellulitis in Stage II lipedema (usually with a component of lymphedema as well), the frequency of cellulitis in Stage II lymphedema is much higher. The time of onset of the "swelling" in lipedema is usually around puberty and 90 per cent of these cases have accompanying diagnoses of hormonal disturbance (thyroid, pituitary, or ovarian). This is usually not the case with primary lymphedema.

A lymphoscintigram may be helpful to differentiate between lymphedema and lipedema, however, there can be conflicting results as lymphedema often occurs to some degree in the later stages of lipedema, probably due to impairment of lymph flow caused by the pressure of fatty tissue. In fact, there are clinical cases of bilateral lower extremity lymphedema in the morbidly obese individual; the onset of the lymphedema occurs after body weight exceeds 350-400 pounds. It is plausible to suspect that the pressure of a large apron of abdominal fat can effectively block lymph flow through the inguinal area causing the lymphedema but there is a difference between these cases and lipedema because obesity does not cause lipedema. Lipedema is caused by a hormonal imbalance resulting in excessive deposition of adipose tissue, most often in the lower extremities (see Figs. 12-21; 12-22) although it can occur in the upper extremities as well.

Treatment and Prognosis.

There is no effective medical treatment for lipedema and the prognosis is guarded; however, significant functional improvements are possible with good program compliance and therapy intervention. Medical management involves treating the hormonal disturbance as effectively as possible and providing nutritional guidance to avoid additional weight gain. Many of these individuals have endured years of ridicule because of their physical appearance and become recluses in their homes, further limiting their activity level. As lipedema progresses and the hypersensitivity increases, they feel less inclined to walk or exercise because of the pain. They inevitably gain more weight due to the inactivity and depression, often finding food their only comfort.

The primary goal of therapy intervention in the person with lipedema is symptomatic relief and realistic improvement of trunk and lower extremity function. Application of the combined lymphedema treatments has shown some success in relieving the pain and hypersensitivity in the lower legs and improving general mobility. Usually, a lower level of compression is needed to support a lipedematous limb, compared to a lymphedematous limb of the same size and girth. This guideline applies to the compression garments as well. These individuals often require more padding under the compression bandages, particularly in the anterior tibial area. They do not tolerate the heavier, denser compression fabrics and usually require a lower grade compression garment than someone with uncomplicated lymphedema. The therapist must remember, however, that later stage lipedema is often accompanied by lymphedema as well, and the treatment and management must take that factor into consideration when recommending exercise and garments.

The main goals of intervention are to decrease pain and hypersensitivity, to decrease the lymphedematous component of the disease, and to assist the individual in maintaining and/or reducing adipose tissue through exercise and nutritional guidance. The compression garments can help to decrease the adipose tissue with exercise and weight loss. The most difficult task is fitting the compression garments. They must be custom made due to the large size of the individual and are often uncomfortable at the waist, particularly when sitting. Making the radical change in daily activity level is most challenging for these individuals. Providing continued support and encouragement is important.

Networking is helpful and is facilitated by offering a support group, even when held on an irregular, informal basis. An hour-long educational meeting, even if only offered three or four times per year can provide a neutral meeting place for people to begin networking. Nothing can compare to the encouragement and hope that an individual with lipedema/lymphedema can derive from seeing and talking with someone else living with the same problem and hearing how others cope on a day-to-day basis. Therapists can learn some of the best guidance on exercise and coping with garments in a group like this.

Lymphedema Therapy

See Also:

Support Group - Lipedema Lipodema Lipoedema

Information Website - Lipedema