Dermatology Online Journal

Lipedema with multiple lipomas
Anabella Pascucci MD¹, Peter J Lynch MD²
Dermatology Online Journal 16 (9): 4

1. Arrowhead Regional Medical Center, Colton, California
2. UC Davis Department of Dermatology, Sacramento, California

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Abstract

Lipedema is an underdiagnosed syndrome of unclear etiology, characterized by symmetric painful enlargement of the buttocks and lower extremities, which spares the feet. This enlargement is caused by the deposition of adipose tissue. It was first described by Allen and Hines in 1940, who observed that it had a female predilection; patients commonly had an associated family history. We describe a patient with classic lipedema and multiple lipomas of her arms and trunk.

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Introduction

Lipedema is a common yet rarely diagnosed syndrome characterized by symmetric enlargement of the buttocks and lower extremities. Allen and Hines first described lipedema in 1940 as being caused by abnormal deposition of adipose tissue in the lower extremities with a peculiar sparing of the feet [1]. Lipedema was further elucidated in an extensive case series by Wold, Hines, and Allen in 1951. They observed that it had a female predilection; only one man was present in their case series of 119 patients. Sixteen percent of those patients had a family history of the condition, including the single male case. Forty percent had associated tenderness in the lower extremities; a smaller percentage had pitting edema. Many had been previously misdiagnosed as having lymphedema [2]. Their findings have been consistently described in scattered case reports throughout the literature. None of the published case reports has described associated lipomas [3, 4, 5, 6, 7]. We describe woman with classic lipedema and multiple lipomas of her arms and trunk.

Case report

A 74-year-old woman noted minor ankle and lower leg enlargement while in high school. By her early thirties, her lower legs, thighs, and buttocks had enlarged to the point that she found it difficult to find a dress size that fit her both on the top and the bottom. Since then she has generally worn two-piece clothing with pants, one or two sizes larger than her shirts. Her leg enlargement has been very slowly progressive to the present time but throughout her life her feet have remained normal in size. From her early twenties she noted that her legs were continuously painful and were extremely tender even to light touch. At about age 30 she began to develop a few semi-firm nodules, one to two inches in diameter, on her arms and subsequently on the trunk and upper thighs. These have also been somewhat tender, though less so than her legs.

She had been examined by multiple physicians, all of whom had diagnosed her problem as that of lymphedema. She was also told that she had lipomas of the arms and trunk, but at no time was a link made with her leg enlargement. It was suggested that she wear compression stockings and that she receive pulse-type leg pumping and massage therapy. The remarkable tenderness of her legs has made her unable to comply with these suggestions. Previous evaluations of her renal, hepatic, and vascular status have revealed no abnormalities.

Figure 1
Figure 1. Enlarged lower extremities sparing the feet.

In 2008 her partner carried out an extensive internet search and suggested to one of us (PJL) the possibility of lipedema as an alternative diagnosis. Examination by PJL at that point revealed notable, abnormal enlargement of the legs from the thighs to the ankle. The enlargement was fairly uniform rather than consisting of confluent individual nodules. The feet below the ankles were normal in appearance and size (Figure 1). No varicosities were present. Marked tenderness to palpation was noted and there was only minimal pitting with fingertip pressure. Approximately 30 individual, smooth-surfaced, semi-firm, subcutaneous nodules (3 to 5 cm in diameter) were found on examination of the arms and trunk.

Her family history revealed that her mother, one grandmother, and one aunt had similar problems with leg enlargement. Another aunt and her father had no similar involvement. She could not recall any history regarding her grandfathers and she has no other close male relatives. The patient and the other affected family members have also had moderate overall obesity. A diagnosis of lipedema with incidental lipomas was established on the basis of her personal history, family history, and physical examination. This diagnosis was subsequently re-confirmed, again on a clinical basis, several months later when she consulted a national expert on lymphedema.

Discussion

Lipedema is an underdiagnosed syndrome of unclear etiology characterized by the deposition of adipose tissue in the buttocks and lower extremities, causing enlargement. Allen and Hines first described it in 1940 as having a female predilection and a family history of similar problems [1]. Lipedema affects bilateral lower extremities symmetrically, sparing the feet. It progresses gradually and is commonly associated with pain [2]. The original case series did not show a particular age of onset, but case reports published since that time seem to show that it commonly begins around puberty. Many patients have been previously misdiagnosed as having lymphedema with years of unsuccessful treatment. They are also often embarrassed by their large legs and have associated anxiety [2-7]. None of the case reports have described coexisting lipomas, as we have described in this patient [2-7]. Lipomas may have a relationship with lipedema; both are characterized by excessive adipose tissue.

Lipedema is a clinical diagnosis that is made by obtaining the typical history, as described above, and supportive physical exam findings. Patients will have tender lower extremities with soft skin, bilateral non-pitting edema, and a sharp demarcation at the ankle caused by the normal skin at the foot. They will also have a negative Stemmer sign. Stemmer sign is positive when the dorsal skin of the foot cannot be pinched because of fluid in the subcutaneous tissues. Lipedema must be distinguished from lymphedema, because it is commonly misdiagnosed as such. Lymphedema exhibits localized swelling caused by lymphatic dysfunction. It typically presents with unilateral pitting edema that may progress to fibrosis; it is not generally painful, and patients may have a history of recurrent cellulitis. Unlike lipedema, patients with lymphedema will have a positive Stemmer sign. They may also have a history of renal, hepatic, or vascular abnormalities. The diagnosis can become complicated in patients with longstanding lipedema who may develop lipo-lymphedema. In lipo-lymphedema, the accumulation of adipose in subcutaneous tissues causes lymphatic dysfunction and subsequent lymphedema [1, 2, 8].

Diagnosis is typically made clinically, but may be aided by diagnostic imaging in difficult cases such as those with lipo-lymphedema. Lymphoscintigraphy and indirect lymphography have been used to search for lymphatic dysfunction and can differentiate between lipedema and lipo-lymphedema [9, 10]. More recently, Magnetic Resonance Lymphangiography (MRL) has been used as a minimally invasive alternative without radiation. MRL can also provide an objective means of following a patient’s progression [11].

Histological analysis of lipedema tissue by Suga et al. showed proliferation of adipose-derived progenitor cells and necrotizing adipocytes. The authors postulated that this was caused by increased adipogenesis leading to hypoxia and adipocyte necrosis [3]. Obtaining a tissue biopsy may be useful to help make the diagnosis of lipedema.

There is a lack of effective treatments for this frustrating syndrome. Diet and lifestyle changes may help obese patients, but rarely provide much improvement [8]. Compression therapy and massage have been extensively used with mixed results. It is likely to be most helpful in patients that have lymphatic dysfunction from progression to lipo-lymphedema. As with our patient, associated tenderness may prevent the continued use of these therapies [12]. Surgical treatments have been tried but results are inconsistent and have associated risks of damaging the lymphatics. Surgical options include debulking by subcutaneous excision, lipectomy, and liposuction [8]. The most important therapy may be what Allen and Hines suggested in their original description of this disorder: emotional support and reassurance [1].

Conclusion

There is still much to learn about lipedema. Its etiology remains unclear and it is not known why it disproportionately affects women. Some have hypothesized its predilection for women may be related to hormones, especially because lipedema commonly begins around puberty. Men afflicted with this disorder often either have cirrhosis or are undergoing hormone therapy for prostate cancer, further supporting hormones as an important factor [4, 11]. Previous case reports describing lipedema do not describe concurrent lipomas [2-7]. We describe the first case of lipedema with multiple lipomas. Lipedema and lipomas may be related; they are both caused by the abnormal accumulation of adipose tissue.

References

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2. Wold LE, Hines EA, and Allen EV. Lipedema of the legs: A syndrome characterized by fat legs and edema. Annals of Internal Medicine 1951; 34(5): 1243-1250. [PubMed]

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9. Weissleder H et al. Value of functional lymphoscintigraphy and indirect lymphangiography in lipedema syndrome. Lymphol 1995; 19: 38-41. [PubMed]

10. Bilancini S et al. Functional lymphatic alterations in patients suffering from lipedema. Angiology 1995; 46(4): 333-339. [PubMed]

11. Lohrmann C, Foeldi E, and Langer M. MR imaging of the lymphatic system in patients with lipedema and lipo-lymphedema. Microvascular Research 2009; 77: 335-339. [PubMed]

12. Macdonald JM et al. Lymphedema, lipedema, and the open wound: The role of compression therapy. Surg Clin N Am 2003; 83: 639-658. [PubMed]

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