Non-Filarial Elephantiasis, a Case Report with Review of the Literature

Clinical Case Report 

Non-Filarial Elephantiasis, a Case Report with Review of the Literature

Corresponding author:  Manuel Lora Gonzalez, Pathology Resident, The University of Kansas Medical Center, USA, Email: Douglas McGregor, Professor, Kansas City VA Medical Center, USA,
Email: douglas.mcgregor@va.orgKeywords: Non-Filarial Lymphedema; Elephantiasis Nostras Verrucosa; Verrucous
Case Report

A A 67 year old male presented to the wound clinic for evaluation of non-healing ulcers and massive edema of the lower extremities. He was an obese male with a history of quadriplegia secondary to septic embolus to the spinal cord. His course had been complicated by recurrent episodes of sepsis, including urosepsis. He routinely followed up at wound clinic for his lower extremity edema and recalcitrant ulcers over a period of years. Five years after his initial spinal cord injury, verrucous plaques began to develop on his inner thighs. The lesions demonstrated a fine scale and were darker than the surrounding tissue. They began as plaques bilaterally and spread slowly over time with formation of satellite lesions followed by coalescence. These lesions appeared to favor areas with prolonged exposure to moisture – remaining on the inner thighs bilaterally and extending to the groin. The primary treatment strategy was moisture reduction with wicking pads changed daily. The lesions did not respond to therapy, and recalcitrant ulcers and osteomyelitis of the left lower leg eventually led to a left above the knee amputation. This did lead to improvement in the verrucous lesions with a moderate reduction in the size of the lesions – presumably related to improved dryness of the area.Biopsy of the plaque demonstrated two pedunculated lesions, confluent at their base, with dermal expansion by fibrocystic cells. There were increased and widened vascular and lymphatic vessels throughout the dermis, but particularly in the superficial dermis. The stroma was variably collagenized and edematous with many stromal cells staining positively for factor XIIIa and CD163. Inflammation was sparse with a mild infiltrate of perivascular lymphocytes. The epidermis was hyperplastic with focal verrucous changes bordering on pseudoepitheliomatous hyperplasia. The changes were considered consistent with verrucous lymphedema.

ENV is a condition characterized by woody fibrosis and often widespread verrucous change of the skin in an area affected by chronic lymphedema. Lymphedema can be categorized as primary (congenital) and secondary (acquired). Worldwide, secondary lymphedema is most often caused by filariasis – however this is not common in Western countries. The term elephantiasis nostras verrucosa was first used in 1969 by Castellani to describe a condition which resulted from recurrent lymphatic blockage related to bacterial infection while other acquired causes of lymphatic obstruction (mycotic, neoplastic, etc.) were termed elephantiasis symptomatica [1]. Subsequently, other authors have included all acquired, non-filarial, non-congenital causes of lymphatic obstruction in the definition of ENV [2-4].

While filariasis is the most common cause of lymphedema worldwide, in Western countries common etiologies include repeated streptococcal lymphangitis/erysipelas, malignancy, trauma, surgery, radiation therapy, chronic venous stasis, scleroderma, and obesity. The blockage or damage of lymphatic channels leads to accumulation of protein-rich fluid in the interstitium. This causes a chronic inflammatory state with subsequent proliferation of fibroblasts, vasculature, and keratinocytes. These changes lead to the firm, cobblestone appearance observed clinically [4-5].

ENV typically includes bilateral involvement of the feet and calves, occasionally extending to the thighs and groin. Although lower extremities are the classic site, ENV can develop the condition wherever chronic lymphedema occurs.
Case table 52.1
Table 1.

All patients demonstrate non-pitting edema with “cobblestone-like” plaques and papules with hyperkeratosis. Larger nodules, ulceration, and changes of chronic venous insufficiency often are identified within the affected area as well. As the condition progresses, the skin becomes more verrucous and increasingly firm [5]. Histologically, the condition is characterized by hyperkeratosis and papillomatosis and often pseudoepitheliomatous hyperplasia. In early stages, there are dilated lymphatics and widened tissue spaces reflecting tissue edema. In later stages, there is extensive fibrosis of the dermis and subcutaneous tissue. Inflammation is sparse to absent with cases occasionally demonstrating a patchy dermal or subcutaneous lymphohistiocytic infiltrate. While hyperkeratosis, dilated lymphatics, and fibrosis are the histologic hallmarks of ENV, all three may not be present in a given biopsy due to the progressive nature of the disease [4, 6].

The clinical differential diagnosis of ENV includes lymphatic filariasis, localized lymphedema, certain deep fungal infections; lipodermatosclerosis, pretibial myxedema, and venous stasis dermatitis (see Table 1). A possible additional consideration includes multiple eruptive angiofibromas – previously reported on the face and trunk. See table 1 for additional discussion. Diagnosis is often clinical with tissue biopsy used in difficult cases although in a review of ENV, Sisto and Khachemoune recommended biopsy of all cases for diagnostic purposes as well as to rule out Stewart-Treves syndrome (angiosarcoma arising in a chronically lymphedematous area) [4,7].

The pathologic findings in our specimen included vasculature proliferation, verrucous epidermal hyperplasia, and a variably edematous and fibrotic dermis. The pathologic differential diagnosis included ENV, localized lymphedema, and multiple eruptive angiofibromas, among others. In our case, serious consideration of multiple eruptive angiofibromas was entertained, however the lesions were coalesced plaques rather than discrete papules and the increase in blood vessels was not dramatic. See table 1 for a complete discussion of the differential diagnosis.

Localized lymphedema is an entity first described in 1995 by Farshid and Weiss [8].

It arises as a single papillomatous plaque, polypoid tumor, or pendulous swelling typically in the ano-genital region or thigh. It is due to localized lymphatic obstruction, often due to obesity, but with reported etiologies identical to ENV. Since the process remains localized, surgical therapy is often curative [9]. The dividing line between ENV and localized lymphedema is blurry as they represent varying degrees of the same process. Our case demonstrated large, coalescing plaques present bilaterally. Although the calf was spared which is unusual in ENV, this remains the most appropriate diagnosis.

ENV is a progressive disease with enlargement and deformity of the involved area over the course of several years. As the tissue expands and becomes increasingly fibrotic, ulcers commonly develop and enlarge and the area becomes colonized with microorganisms. This often leads to morbid infections including osteomyelitis and septic arthritis which may require amputation. As mentioned previously, Stewart-Treves syndrome is an uncommon development but is associated with a poor prognosis [4, 10].

Initial treatments include massage, compression stockings, and pneumatic compression pumps [11]. Oral retinoids have been used with moderate success and antimicrobial therapy is often required [4, 12]. If medical and compression therapies are not effective, amputation or debridement of the affected area can be performed [13]. The disease is difficult to treat with occasional positive outcomes using each of the therapy strategies. Currently however, there is no reproducibly effective treatment option.

Case fig 52.1

Figure 1. Lower extremity photograph showing generalized edema, coalescent verrucous plaques with dark pigmentation (A) and focal yellow crust (B).

Case fig 52.2

Figure 2. Hematoxylin and Eosin stained sections showed hyperkeratosis, papillomatosis, pseudoepitheliomatous hyperplasia, fibrosis of the dermis and vascular proliferation with sparse perivascular and interstitial inflammation.

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