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Dermatology Online Journal

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Elephantiasis nostras verrucosa: an atypical presentation following intrapelvic lymphoma


Elephantiasis nostras verrucosa is a progressively debilitating and disfiguring disease commonly presenting with verrucous, cobblestone-like papules, nodules, or plaques with nonpitting edema in the lower extremities. Histopathology is marked by hyperkeratosis and dermal or subcutaneous fibrosis as a result of chronic lymphedema. Risk factors include obesity, recurrent cellulitis, chronic venous insufficiency, congestive heart failure, scleroderma, radiation, trauma, and tumors. We report a 72-year-old man who presented to the dermatology clinic for an 11-year history of edematous legs, occasionally associated with ulcerations. The findings developed within a year of intrapelvic non-Hodgkin lymphoma and progressed gradually over 10 years after lymphoma remission. Physical examination revealed atypical features including compressible cysts and pitting edema extending from the lower legs to the thighs bilaterally. The patient was noncompliant for the recommended compressive devices and the condition progressively worsened over the course of 7 months of follow-up. Early interdisciplinary management using compressive devices and a lymphatic pump are recommended. Underlying causative factors should be assessed with regular follow-up to optimize treatment outcomes.

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