A Case of Chronic Elephantiasis Nostra Verrucosa Highlighting the Progression of Chronic Lymphedema

TL;DR

This paper presents a rare case of chronic lymphedema called elephantiasis nostra verrucosa in a patient with multiple health issues, emphasizing the challenges in managing and preventing this condition.

Contribution

The paper contributes a detailed case report of ENV in a patient with ESRD, morbid obesity, and cellulitis, highlighting management strategies and the need for early intervention.

Findings

ENV is a rare, nonfilarial chronic lymphedema often linked to obesity and venous insufficiency.

Treatment for ENV is primarily conservative, with limited evidence for retinoids or surgery.

Early intervention and patient education are crucial in preventing ENV progression.

Abstract

Elephantiasis nostra verrucosa (ENV) is a rare form of secondary, nonfilarial chronic lymphedema, typically linked to conditions such as morbid obesity, congestive heart failure, and chronic venous insufficiency (CVI). Bacterial infection is thought to initiate a vicious cycle of inflammation, limb swelling, and fibroblast proliferation that results in the “cobblestone” appearance of the affected extremity.

A 67-year-old male with a past medical history of end-stage renal disease (ESRD) on hemodialysis, morbid obesity, type 2 diabetes mellitus, and ENV presented to our ED with cellulitis of his right lower extremity after minimal trauma to his leg. The patient had a BMI of 60.7 kg/m², ENV of the bilateral lower extremities with cellulitis of the right leg, and annular psoriasis diffusely involving the bilateral arms, abdomen, back, head, and neck. Management of the right leg cellulitis included a CT scan to evaluate for osteomyelitis, IV vancomycin and cefepime for broad-spectrum coverage, and consultations with Nephrology and Dermatology to coordinate hemodialysis and antibiotic dosing in the setting of ESRD, and management of annular psoriasis, respectively. Patient education on hygiene for the lower extremities and fistula site was also provided. The patient was discharged after 3 days with outpatient Dermatology follow-up established.

ENV, although rare, presents a significant challenge to patients and physicians. Much of the literature on ENV derives from case reports, with limited data to elucidate what specifically provokes its development. This scarcity of literature creates challenges in incorporating evidence-based treatment regimens. As seen in our patient’s case, recommendations for ENV are typically conservative measures, such as weight loss, infection prevention, and lymphatic pumps or compression garments. The use of retinoids and surgical intervention has also been reported, with minimal improvement. Our case highlights the importance of preventing conditions such as ENV through early intervention and patient education.

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