A Case of Elephantiasis Nostras Verrucosa

Abstract

Elephantiasis nostras verrucosa (ENV) is a rare clinical condition associated with chronic non-filarial lymphedema caused by bacterial or non-infectious lymphatic obstruction. A variety of etiologies, including infection, tumor obstruction, trauma, radiation, chronic venous stasis, congestive heart failure, and obesity, can lead to chronic lymphatic obstruction and edema. Mossy papules, plaques, and cobblestone-like nodules are clinically impressive features of ENV, but biopsy reveals only moderately abnormal findings such as pseudoepitheliomatous hyperplasia, dilated lymphatic spaces, fibrous tissue hyperplasia, and chronic inflammation. We present a case of ENV in a 67-year-old man with a 10-year history of multiple nodules and verrucous plaques on both feet. Microbiology ruled out a filarial infection. Nodule biopsy revealed pseudoepitheliomatous hyperplasia, marked dermal fibrosis, and a chronic inflammatory infiltrate. No evidence of carcinoma was identified. Both venous stasis and recurrent cellulitis could contribute to the dermal fibrotic changes of the lesions. However, before the recurrent cellulitis, he did not have any nodular lesions on his feet despite a 10-year history of venous disease. Therefore, this case suggests that venous stasis alone cannot produce the fibrotic nodular lesions of ENV. (Ann Dermatol 21(3) 326∼329, 2009)

Sweet syndrome with pseudocarcinomatous hyperplasia: A case report and review of the literature

“…PEH is widely recognized to occur in the context of healing surgical scars and in chronic lymphedema (elephantiasis nostras verrucosa) . Multiple reports have described PEH as part of a hypersensitivity reaction to tattoos; the observation of keratoacanthoma arising within red tattoos may represent a related phenomeon .…”

Excision of Elephantiasis Nostras Verrucosa Lesions in a Patient With Hereditary Lymphedema: Case Report and Review of the Literature

“…Although previously thought to be secondary to an infectious etiology, ENV is now understood to be a sequela of chronic lymphedema and can be secondary to a number of noninfectious causes, including neoplasia and trauma (19)(20)(21). A rare disease, ENV is characterized by progressive cutaneous hypertrophy and nonpitting edema that often gives a mossy and cobblestoned appearance to the affected extremity or body region (22).…”

“…A rare disease, ENV is characterized by progressive cutaneous hypertrophy and nonpitting edema that often gives a mossy and cobblestoned appearance to the affected extremity or body region (22). Histologically, ENV is characterized by pseudoepitheliomatous hyperplasia with dermal fibrosis; chronic inflammatory infiltrates are often present (21). Although the risk factors remain incompletely elucidated, morbid obesity with superimposed venous insufficiency is present in most patients.…”

A Case of Exuberant Elephantiasis Nostras Verrucosa

“…1,3 Early recognition and treatment of this peculiar entity is the key to achieve a satisfactory result. Elephantiasis nostras verrucosa (ENV) is a rare deforming disorder associated with chronic nonfilarial lymphedema.…”