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Why Are My Legs Swollen?

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Issue Number: Volume 21 - Number 4 - April 2013 [1]

Section: PhotoDX

Topics: PhotoDx

Citation:

Norman RA, Aun JF. Why are my legs swollen? Clinical Geriatrics. 2013;21(4):20,22-23.

Authors:

Robert A. Norman, DO, MPH Jessica Fides Aun

Dr. Norman is in private practice in Tampa, FL. He is also associate professor, University of Central Florida School of

Medicine, Orlando, and at Nova Southeastern School of Medicine, Davie, FL. Ms. Aun is a third-year student of osteopathic

medicine at Lake Erie College of Osteopathic Medicine, Bradenton, FL.

An overweight 66-year-old man presented with a chief concern of scaly, swollen legs that he reported became numb as the day

progressed. The patient said he observed the condition started at his ankles, which were red and itchy, approximately 2 years

prior to his current presentation. It gradually moved upward on both legs and progressively worsened. He noted that his legs

sometimes oozed with a watery discharge, but he reported no pain in his extremities. The numbness subsided when he
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elevated his legs, he said, and triamcinolone cream helped to relieve his symptoms. The patients medical history included

obesity, hypertension, type 2 diabetes, cirrhosis, anemia, and deep venous thrombosis. He reported shortness of breath when

he walked around, which was relieved upon resting. The patient had a 40-pack-year history of smoking, but he had quit

smoking 11 years prior to his current presentation. He had not recently traveled out of the United States. Upon physical

examination, the patients lower extremities were swollen with diffuse, scaly, erythematous papules (Figures 1 and 2). There

was evidence of nonpitting peripheral edema. Deep fissures were present around his ankles. The patient was taking numerous

medications, including furosemide, lisinopril, tramadol, and famotidine.

[2]

Based on the photographs and the case description, what is your diagnosis?

A. Lymphatic filariasis

B. Podoconiosis

C. Elephantiasis nostras verrucosa

D. Lipedema verrucosa

E. Chronic obesity lymphedematous mucinosis

Click on the next page for the answer and a discussion>>

Diagnosis: Elephantiasis nostras verrucosa (C)

Discussion

Elephantiasis nostras verrucosa (ENV) is a rare clinical entity associated with nonfilarial lymphedema.1 The term elephantiasis

is commonly used to describe a body part that is enlarged and disfigured due to edema and fibrosis; but historically, the term

refers to swelling of the legs caused by parasitic infection of various species of roundworms, the most common of which in

humans is Wuchereria bancrofti.1 The term nostras was added to the diagnosis in 1934 to distinguish ENV from other

lymphedematous disorders of temperate regions not caused by filariasis.1 ENV is currently understood as a dermatological

complication caused by bacterial or non-

infectious lymphatic obstruction, which has numerous etiologies, including tumor obstruction, radiation, obesity, congestive

heart failure (CHF), and trauma.1 Swelling typically occurs in the subcutaneous layer.2 Cellulitis and lymphangitis are common

complications of lymphedema that cause distinguishing skin changes of ENV, including papillomatosis, hyperkeratosis,

increased creases, and thickening of the skin.2 Cobblestone-like verrucous nodules frequently appear along with lichenified

skin and hyperpigmentation.3,4

The patients history, physical examination, and presence of typical cutaneous lesions usually suffice to make a diagnosis of


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ENV.4 In our case patient, we suspected ENV based on physical examination of the patients lesions, his medical history of

obesity, and his statement that he had not recently traveled. Further investigation was needed to see if CHF also contributed to

his condition. Our patient did not have a history of CHF; however, we suspected that he may have had undiagnosed CHF

because CHF is often contributory to this type of lymphatic obstruction, and he had several signs and symptoms of CHF. It is

not known if the patient followed up with his primary care provider to further investigate possible CHF.

Pathology Findings

Histopathologic examination of biopsy findings and imaging techniques, such as isotope lymphography, lymphangiography,

computed tomography, and magnetic resonance imaging, may assist in the differential diagnosis of ENV, especially to rule out

malignancy.1-3 Pseudoepitheliomatous hyperplasia, dilated lymphatic spaces, and fibrosis are characteristic findings of ENV.1A biopsy was not conducted for our case patient because his history and physical examination provided enough basis to make

a diagnosis of ENV.

Approaches to Treatment

If left untreated, ENV can cause severe disfiguration to the involved limb.5 When treating ENV, it is imperative to address the

edema as soon as possible to help prevent bacterial infection and fibrosis. Treatment to help stimulate lymph flow is important

in helping control lymph formation and drainage.2 Physical forms of treatment include exercise, compression with hosiery,

manual massage, pneumatic compression, and elevation of the affected limb.1,2 If indicated, antibiotics should be given

immediately to prevent bacterial infections from escalating.2 In some cases, prophylactic antibiotics may be prescribed to

those with recurring infections.2 There is some evidence that oral etretinate improves lymphedema, hyperkeratosis, and

verrucous lesions while also flattening the papillomatous nodules that are prominent in this condition.6 For some patients whoselimbs have become significantly enlarged, surgical removal of excessive tissue and fluid may be warranted.2,5

Ruling Out the Other Diagnoses

What follows is a brief review of some of the other possible diagnoses, which were ruled out for our case patient.

Lymphatic Filariasis

Lymphatic filariasis is one of the most common causes of secondary lymphedema occurring in the tropical and subtropical

regions of the world.7 Several of the roundworm species (nematodes) known to cause lymphatic filariasis are Wuchereria

bancrofti, Brugia malayi, and Brugia timori.1,7Although the pathology is not fully understood, evidence shows that nematodes

may cause abnormalities in the integrity of the lymphatic vessels and prevent appropriate functions, such as lymph production

and drainage.7 The lymphedema in lymphatic filariasis mainly affects the lower extremities but can also involve the arms, male

genitalia, and breasts.3,8 Clinical diagnosis of lymphatic filariasis are commonly made in endemic regions through examination

of the affected limb.8 Other tests used to determine filarial infestation include night blood examinations to detect microfilariae

and the immunochromatographic card test for the filarial antigens.8

Podoconiosis

In areas of the world where there are high amounts of red clay soil of volcanic origin, podoconiosis, a type of noninfectious

elephantiasis, is common.9 Case reports of manual laborers in agricultural regions show that this disease presents more

frequently in younger persons, typically in their second or third decade of life.10,11 It is believed that this type of bilateral

nonfilarial elephantiasis is an inflammatory response in the lymphatics caused by long-term exposure to particles in the red clay

soil.9 Almost all patients with pondoconiosis have adenolymphangitis (red, hot legs and swollen, painful groin). 10 The condition

is rare, as it is most often prevented by wearing socks and shoes in these regions of the world, practicing regular foot hygiene,and using emollients.10

Lipedema

The chronic, irregular, and bilateral deposition of adipose in the lower limbs, between the pelvic crest and ankle, is known as

lipedema.12 The legs of an individual with lipedema are described as being in the shape of an Egyptian column.13 Commonly

misdiagnosed as lymphedema, this disease of abnormal lipid metabolism usually does not involve the feet or the upper

extremities.12 Occasionally, subcutaneous nodules can be palpated, which results from an accumulation of adipose tissue.12

Often, there is a known family history of lipedema and it is first diagnosed in puberty. Lipedema is more commonly seen in men

than in women and can worsen during pregnancy.13 This condition presents with a negative Stemmers sign (a thickened skin

fold at the base of the second toe or second finger), is characterized by skin that is cool to the touch but can be painful, and


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typically coexists with edema and changes in plantar support.13

There are three distinguishable stages of lipedema. During the first stage, the skin is still soft with nodules equally dispersed

throughout the subcutaneous tissues.12 In the second stage, the nodules are no longer spaced evenly throughout the

subcutaneous tissue, and the skin has the characteristic look of an orange peel.12 The third stage is marked by subcutaneous

tissue that extends beyond the skin of the knees and thighs.12 Increased fragility of the nearby vessels can cause petechiae as

well as hematomas.12 The irregular lipid metabolism involves hyperplasia of the individual fat cells, affecting both distribution

and storage.12 A histological examination of lipedema reveals necrosing adipocytes along with progenitor cells originating

from adipose tissue.13

Chronic Obesity Lymphedematous Mucinosis

Pretibial myxedema is often related to thyroid disorders; however, when mucin deposition is observed in euthyroid individuals,

a diagnosis of obesity-associated lymphedematous mucinosis may be suspected.14 It is suggested that the cause of mucin

deposition in pretibial skin is stasis of the lymphatic fluid.14 Patients with chronic obesity lymphedematous mucinosis are

typically morbidly obese and have painless, pitting edema in their lower extremities, rarely affecting the upper extremities.14,15

This condition first presents as waxy, pink or yellow cutaneous nodules, with gradual thickening of the skin.15 A histological

examination shows separation of collagen bundles and effacement of the rete ridges with deposition of mucin around vessels

and superficial papi llary dermis.14

References

1. Mortimer PS. Swollen lower limb-2: lymphoedema. BMJ. 2000;320(7248):1527--1529.

2. Berngard SC, Narayanan V. Elephantiasis nostras verrucosa of the pannus. J Gen Intern Med. 2011;26(7):810.

3. Yang YS, Ahn JJ, Haw S, Shin MK, Haw CR. A case of elephantiasis nostras verrucosa.Ann Dermatol. 2009;21(3):326-

329.

4. Guarneri C, Vaccaro M. What is your call?: cobblestone-like skin. Elephantiasis nostras verrucosa. CMAJ.

2008;179(7):673-674.

5. de Godoy JM, Azoubel LM, de Fatima Guerreiro Godoy M. Surgical treatment of elephantiasis of the feet in congenital

lymphedema to facilitate the use of a compression mechanism. Int J Gen Med. 2010;3:115-118.

6. Zouboulis CC, Bicz S, Gollnick H, et al. Elephantiasis nostras verrucosa: beneficial effect of oral etretinate therapy. Br J

Dermatol. 1992;127(4):411-416.

7. Bennuru S, Nutman TB. Lymphatics in human lymphatic filariasis: in vitro models of parasite-

induced lymphatic remodeling. Lymphat Res Biol. 2009;7(4):215-219.

8. Shenoy RK. Clinical and pathological aspects of filarial lymphedema and its management. Korean J Parasitol.

2008;46(3):119-125.

9. Molla YB, Tomczyk S, Amberbir T, Tamiru A, Davey G. Podoconiosis in East and West Gojam Zones, Northern Ethiopia.

PLoS Negl Trop Dis. 2012;6(7):e1744.

10. Alemu G, Ayele F, Daniel T, Ahrens C, Davey G. Burden of podoconiosis in poor rural communities in Gulliso woreda,

West Ethiopia. PLoS Negl Trop Dis. 2011;5(6):e1184.

11. Sikorski C, Ashine M, Zeleke Z, Davey G. Effectiveness of a simple lymphoedema treatment regimen in podoconiosis

management in Southern Ethiopia: one year follow-up. PLoS Negl Trop Dis. 2010;4(11):e902.

12. Shin BW, Sim YJ, Jeong HJ, Kim GC. Lipedema, a rare disease.Ann Rehabil Med. 2011;35(6):

922-927.

13. Godoy Mde F, Buzato E, Brigidio PA, Pereira de Godoy JM. Is lymphostasis an aggravant of lipedema? Case Rep

Dermatol. 2012;4(3):222-226.

14. Tokuda Y, Kawachi S, Murata H, Saida T. Chronic obesity lymphoedematous mucinosis: three cases of pretibial


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mucinosis in obese patients with pitting oedema. Br J Dermatol. 2006;154(1):157-161.

15. Stewart G, Kinmonth JB, Browse NL. Pretibial myxoedema.Ann R Col l Surg Engl. 1984;66(6):

391-395.

The authors report no relevant financial relationships.

[3] [4]

[5]

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