Diagnosis: Elephantiasis nostras verrucosa

Elephantiasis nostras is a rare cutaneous response to chronic secondary, nonfilarial lymphedema. Our patient had elephantiasis nostras verrucosa (ENV), a hard subtype that can occasionally coexist with ENV glabra (smooth, soft). Usually found on the lower extremities and genitalia, ENV has rarely been reported on the arms, chest, pendulous abdomen, and external ear after recurrent bouts of pustular psoriasis.1

Pathogenesis of ENV involves lymphatic obstruction, impaired drainage, and stasis of protein-rich lymph fluid, with development of chronic interstitial lymphedema. The resulting inflammation and fibrogenesis eventuates in dermal fibrosis and overlying epidermal changes. Chronic venous stasis is a common coexisting and aggravating factor.2 


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Etiologies of ENV lymphedema include disruption of the lymphatic channels; trauma; surgery; fibrotic obliteration by morphea, scleredema, or radiation; malaria or schistosomiasis; malignant infiltration; chronic congestive heart failure; portal hypertension; and obesity. Classic elephantiasis tropica has been linked to recurrent Streptococcus metamyceticus lymphangitis, but ENV is only in part attributed to a microbial infection.2 The pathogen enters through insignificant trauma, e.g., interdigital fissures of tinea pedis, and with each episode of lymphangitis, disrupts the lymphatics, increasing lymphedema and predisposing the extremity to further infection. 

Histopathologic changes include pseudoepitheliomatous hyperplasia with hyperkeratosis, parakeratosis, acanthosis, and papillomatosis. Within the diffusely edematous papillary and reticular dermis are many dilated lymphatic spaces. Chronic inflammatory infiltrate is eventually replaced by fibrosis.2 

Diagnosis of ENV is largely clinical. Lymphangioscintigraphy and, rarely, lymphangiography—along with ultrasound, CT, or MRI of the extremities, abdomen, and pelvis—can help exclude other causes, define the etiology of lymphedema, and rule out malignant obstruction.2 Distinction from papillomatosis cutis carcinoides (verrucous carcinoma) requires biopsy.2 

Treatment focuses on normalizing chronic skin changes as well as alleviating the lymphedema and managing its underlying etiology. Leg elevation, massage, elastic bandages, and intermittent pneumatic compression decrease stasis. Coumarin–related compounds, available outside the United States, decrease the protein–rich interstitial lymph fluid via their proteolytic effect.2 Therapeutic and prophylactic topical and systemic antibiotics decrease the number of lymphangitic episodes, and topical and systemic antifungals for tinea pedis heal the portal of entry. Emollients and keratolytics, including ammonium lactate, salicylic acid, and acetic acid preparations, help alleviate secondary epidermal changes. Topical and systemic retinoids, such as tazarotene (Tazorac) and acitretin, help normalize keratinization and decrease inflammation and fibrosis. Treatment must also manage any underlying systemic etiology for lymphedema. Surgical approaches range from local debridement to restoration of lymph flow using lymphatic or lymphovenous anastomoses or lymphatic transplantation.2 

Our patient was treated with piperacillin/tazobactam (Zosyn) for presumed secondary bacterial infection, leg elevation, ammonium lactate and urea 40% creams twice daily, and tazarotene 0.1% gel nightly. Consideration was given to oral acitretin therapy and Unna boot placement (since his peripheral arterial perfusion was not compromised). Nevertheless, he developed bleeding from one of the extremities. The advanced stage of the disease also raised concerns of necrosis in the affected extremities and the infectious risk that posed. Eventually, he underwent bilateral above-the-knee amputation, without complications. 

At the time of this writing, Dr. Dudelzak was a resident in the Department of Medicine, Division of Dermatology, at the Medical College of Georgia in Augusta, where Dr. Sheehan is an assistant professor.

 

References

1. Vaccaro M, Borgia F, Guarneri F, Cannavo SP. Elephantiasis nostras verrucosa. Dermatol. 2000;39:764-766.  
2. Schissel DJ, Hivnor C, Elston DM. Elephantiasis nostras verrucosa. Cutis. 1998;62:77-80.