Elephantiasis Nostras Verrucosa Swelling with Verrucose Appearance of Lower Limbs
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Web exclusive | Case Report Elephantiasis nostras verrucosa Swelling with verrucose appearance of lower limbs Fang-Yih Liaw MD Ching-Fu Huang MD Yu-Cheng Wu MD Bai-Yao Wu MD Editor’s kEy points lephantiasis nostras verrucosa (ENV) is a rare form of chronic lymphedema that • Elephantiasis nostras verrucosa (ENV) Ecauses progressive cutaneous hypertrophy. It can lead to severe disfiguration is a rare form of chronic lymphedema of body parts with gravity-dependent blood flow, especially the lower extremities. and can be mistaken for other diseases, Various factors can cause obstruction of the lymphatic system and result in ENV. such as venous stasis dermatitis, filariasis, Clinically, ENV is characterized by nonpitting edema and superimposed hyperkera- lipedema, chromoblastomycosis, lipoder- totic papulonodules with a verrucose or cobblestone-like appearance. (Early-stage matosclerosis, and pretibial myxedema. lesions might exhibit pitting edema; late-stage lesions exhibit nonpitting edema.) It Several factors, including neoplasms, needs to be differentiated from pretibial myxedema, filariasis, lipedema, chromo- trauma, radiation treatment, congestive blastomycosis, lipodermatosclerosis, and venous stasis dermatitis (Table 1).1-5 heart failure, obesity, hypothyroidism, chronic venous stasis, and filarial infec- table 1. Differential diagnosis of elephantiasis nostras verrucosa tion can cause secondary lymphedema. Diagnosis CLiniCaL FEatuREs Venous stasis Pitting edema, erythematous to brownish pruritic patches with • Nonpitting edema and superimposed dermatitis dilated superficial veins over the medial lower leg1-3 verrucose, cobblestone-like nodules on Lipedema Early age of onset; positive family history; typically affects body parts with gravity-dependent blood overweight women; abnormal accumulation of subcutaneous flow are characteristic of ENV. Protein- fat in lower limbs and buttocks; always symmetric and bilateral; rich fluid accumulation and recurrent feet not affected1,4,5 infection cause ENV, resulting in a vicious Lipodermatosclerosis Related to venous stasis, hyperpigmentation, and nonpitting cycle. edema with subcutaneous fibrosis. Characteristic “inverted wine bottle” appearance: swelling of proximal parts and fibrosis of • Early diagnosis and treatment the distal parts of the lower limbs1,4 can reduce the morbidity and mortality Pretibial myxedema Caused by hyperthyroidism. Mucin accumulation of the dermis of ENV. resulting in nonpitting edematous papulonodules or plaques over anterior surface of the legs and dorsal aspect of the feet1,4 points dE rEpèrE du rédactEur Filariasis Infection by the parasite Wuchereria bancrofti. History of travel • L’éléphantiasis nostras verruqueux (ENV) to an endemic tropical area. Obstruction of lymphatic ducts est une forme rare de lymphœdème et causes secondary bacterial infection. Localized lymphedema in peut être confondu avec d’autres mala- the lower legs and genitals1,4 dies comme la dermatite de la stase vein- Chromoblastomycosis Chronic fungal infection; fungus is commonly found in soil. euse, la filariose, le lipœdème, la chromo- Infection through minor trauma of skin. Verrucose papulonodules blastomycose, la lipodermatosclérose et and plaques. Cultures are positive for fungus4 le myxœdème prétibial. Divers facteurs, Data from Baird et al,1 Yang et al,2 Guarneri and Vaccaro,3 Sisto and Khachemoune,4 and Kerchner y compris les tumeurs, les traumatismes, et al.5 la radiothérapie, l’insuffisance cardiaque congestive, l’obésité, l’hypothyroïdie, la It is important for family physicians to recognize the underlying conditions, stase veineuse chronique et l’infection clinical presentation, and differential diagnosis of ENV, and to be familiar with filarienne, peuvent causer un lym- its treatment. Therefore, we present a case of ENV in a 74-year-old man who phœdème secondaire. had congestive heart failure, diabetic nephropathy, and obesity. • Parmi les caractéristiques de l’ENV Case figurent un œdème ne prenant pas le godet A 74-year-old man presented to the outpatient department with progressive swell- et des nodules verruqueux superposés ing and numerous protruding masses bilaterally on the lower limbs, which he had ressemblant à des pierres sur les parties had for the past year. He also complained of worsening dyspnea on exertion. His du corps où la circulation sanguine est influencée par la gravité. L’accumulation medical history was relevant for congestive heart failure, type 2 diabetes mellitus de liquides riches en protéines et l’infection with nephropathy, and morbid obesity (body mass index 40.4 kg/m2). His surgical, récurrente causent l’ENV, entraînant un travel, and family histories were unremarkable. cercle vicieux. On physical examination, his lower extremities showed pitting edema; licheni- fication; indurated, cobblestone-like papulonodules; and plaques Figure( 1). • Le diagnostic et le traitement précoces Kaposi-Stemmer sign (ie, the inability to pinch the dorsal aspect of the skin at the peuvent réduire la morbidité et la base of the second toe) was present (Figure 2). mortalité de l’ENV. Vol 58: october • octobre 2012 | Canadian Family Physician • Le Médecin de famille canadien e551 Case Report Laboratory evaluation of the patient’s samples (Figure 3). Based on these clinical and imaging find- revealed normal results for the complete blood count ings, the final diagnosis was ENV. and liver function tests. Thyroid function and C-reactive The patient received intravenous furosemide and protein levels were found to be in the normal range. heparin to control edema and deep vein thrombosis. Abnormal laboratory results included a blood urea In addition, we also encouraged him to increase nitrogen level of 17.493 mmol/L (normal range 1.8 ambulation, elevate his legs, and reduce his body to 7.2 mmol/L) and a creatinine level of 221 µmol/L weight. However, his condition did not substantially (normal range 50 to 110 µmol/L). Doppler sonography improve owing to poor compliance. We consulted a of the deep veins bilaterally in the lower limbs revealed general surgeon to manage the deep vein thrombosis, partial thrombi bilaterally in the common femoral vein but the patient refused any intervention. His general and in the right deep femoral vein. Magnetic resonance condition became worse and he was intubated and imaging revealed generalized swelling in the subcuta- followed in the intensive care unit owing to respira- neous layer and lymphedema of the bilateral lower legs tory failure. He died 5 days later. Discussion Figure 1. Elephantiasis nostras verrucosa: A) Elephantiasis nostras verrucosa is an exaggerated form of Verrucose, cobblestone-like papulonodules and plaques secondary nonfilarial lymphedema. The term elephantiasis bilaterally over the lower legs. B) Detailed view of is used to describe a body part that becomes enlarged papulonodules and plaques on the right lower limb of and disfigured due to edema and fibrosis of the skin. the patient. Several conditions that block lymphatic drainage can induce lymphedema, including neoplasms, trauma, radia- tion treatment, congestive heart failure,1 obesity, hypo- thyroidism,6 chronic venous stasis,2 and filarial infection. Figure 3. Magnetic resonance imaging of lower limb lymphedema: Note the thickening of the skin and honeycomb appearance of the subcutaneous tissue (box). Figure 2. Kaposi-stemmer sign e552 Canadian Family Physician • Le Médecin de famille canadien | Vol 58: october • octobre 2012 Case Report The pathogenesis of ENV is still unclear. It is conceiv- or systemic retinoids.1 Owing to the teratogenicity of able that first the lymphatic channels are damaged and systemic retinoids, it is important to provide contracep- blocked due to one or more of the above-mentioned tion to female patients before treatment. Patients should conditions, and excessive protein-rich fluid accumulates also receive careful monitoring of serum lipids and liver in the dermis and subcutaneous tissues. Second, the function. Surgical intervention can be considered in protein-rich fluid decreases oxygen tension and might recalcitrant cases when the response to medical treat- decrease the immunity of the skin. Third, poor immunity ment is poor.11 However, unsatisfactory outcomes are increases the skin’s susceptibility to infection by micro- common in the management of advanced stages of ENV. organisms. Finally, there is swelling, fibrosis, and dis- The expected survival for a patient with ENV is based figuration of the affected areas.4 Hence, a vicious cycle on the severity of lymphedema, underlying diseases, and begins, as the underlying conditions predispose the skin other contributing factors. Early diagnosis and early inter- to microbial infections. vention in the vicious cycle will render a better outcome. Elephantiasis nostras verrucosa is commonly observed in gravity-dependent parts of the body, espe- Conclusion cially in the lower extremities. In addition, other sites Different conditions can cause ENV. Determining the including the upper extremities, abdomen, buttocks, face, underlying causes and initiating treatment during the or scrotum might be involved.7-9 Elephantiasis nostras early stage can improve the expected survival. History verrucosa usually begins at the dorsal aspect of the foot taking and physical examination are sufficient to diag- and then progresses to the proximal parts of the limbs. In nose ENV. Laboratory tests and imaging studies will the beginning, the lesion presents as mild and persistent provide more