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.

Keywords: Cellulitis; Elephantiasis; Elephantiasis nostras verrucosa; Venous stasis.

Fig. 1

(A) Pink or brown hard papules and nodules on both toes and toe webs. (B, C) Plaques with a moss-covered appearance surround the heel and outer edge of both feet.

Fig. 2

(A) Histologic examination demonstrates hyperkeratosis, papillomatosis, and pseudoepitheliomatous hyperplasia of the epidermis and marked dermal fibrosis (H&E, ×40). (B) Dilated lymphatic channels are observed in the upper dermis (D2-40, ×100).